Anemia

Anemia

Anemia Part 1

How long do sickle cell anemia patients receive penicillin prophylaxis?

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Age 2 months to 5 years

Anemia Part 1
Anemia

What kidney problems can occur in sickle cell anemia patients with low blood pressure?

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Renal papillary necrosis

Anemia
Anemia

What is the iron, TIBC, ferritin pattern for anemia of chronic disease?

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Low iron, low TIBC, high ferritin

Anemia
Anemia

What is the purpose of the hexose monophosphate shunt?

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To ensure that there is enough glutathione available to maintain a low oxidative stress environment

Anemia
Anemia

What are 5 important molecules for the glycolysis and hexose monophosphate shunt pathway?

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ATP, glutathione, reduced NADH, reduce NADPH, 2,3-BPG

Anemia
Anemia

What are the most important hemolytic processes in the red blood cell?

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Glycolysis and hexose monophosphate shunt

Anemia
Anemia

What red blood cell surface antigen deficiency leads to acanthocytes?

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Rh deficiency

Anemia
Anemia

What genetic syndrome leads to Acanthocytes on a peripheral smear?

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A- beta-2–lipoproteinemia
Ataxia retinitis pigmentosa

Anemia
Anemia

What is the syndrome that leads to hemolytic anemia in liver cirrhosis?

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Zeive Syndrome

Anemia
Anemia

What is the mechanism of acanthocytosis?

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Cholesterol movements from lipoproteins of red blood cell membranes and spleen remodels to red
blood cell

Anemia
Anemia

In what disease is acanthocytosis seen most of the time?

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Liver disease

Anemia
Anemia

What are secondary causes of stomatocytosis?

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Smear preparation artifact, alcohol, liver disease, biliary disease, cancer, heart disease

Anemia
Anemia

What is the risk of having a splenectomy in hereditary stomatocytosis ?

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Deep venous thrombosis

Anemia
Anemia

What is a specific test for hereditary Pyropoikylocytosis?

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Easy RBC fragmentation at 45-50°C

Anemia
Anemia

What is the MCV for hereditary Pyropoikylocytosis?

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50-60fL

Anemia
Anemia

What percentage of red blood cells need to have an elliptical shape on a peripheral smear to be 100%
sensitive for hereditary elliptocytosis?

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At least 60%

Anemia
Anemia

Which proteins are mutated in hereditary elliptocytosis?

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Spectrin and Protein 4.1

Anemia
Anemia

What is the most severe form of hereditary hemolysis?

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Hereditary Pyropoikylocytosis – autosomal recessive

Anemia
Anemia

What is the inheritance pattern of hereditary elliptocytosis?

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Autosomal dominant

Anemia
Anemia

What are the 3 different types of hereditary elliptocytosis?

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Common, Spherocytic, Southeast Asian Ovalocytosis

Anemia
Anemia

What are the 3 different types of hereditary elliptocytosis?

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Common, Spherocytic, Southeast Asian Ovalocytosis

Anemia
Anemia

How should children with hereditary spherocytosis be prophylaxed ?

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Penicillin for 5 years

Anemia
Anemia

What are the 2 surgical options for hereditary spherocytosis?

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Partial and full splenectomy

Anemia
Anemia

Which osmotic test has high-sensitivity for hereditary spherocytosis?

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Osmotic gradient Ektacytometry

Anemia
Anemia

Which test is specific for hereditary spherocytosis?

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Eosin-5-Maleimide

Anemia
Anemia

What CBC differences are seen with hereditary spherocytosis?

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Elevated mean corpuscular hemoglobin concentration

Anemia
Anemia

What would a mass seen on imaging in hereditary spherocytosis likely indicate?

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Extra medullary hematopoiesis

Anemia
Anemia Part 2

What are the 2 reasons there is splenomegaly for beta thalassemia?

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Increased red blood cell destruction and extra medullary hematopoiesis

Anemia Part 2
Anemia

Which genetic changes lead to Beta+ genotype?

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Point mutations lead to a spectrum of protein synthesis loss

Anemia
Anemia

What genetic changes lead to a Beta-0 genotype?

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Insertion or deletion of the promoter region leading to complete loss of synthesis

Anemia
Anemia

What defines Cooley’s anemia?

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Beta-0-0 = 99 – 100% of Cooley cases

Anemia
Anemia

Which geographic region has beta thalassemia?

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Mediterranean and southeast Asia

Anemia
Anemia

What are the 3 families of intrinsic hemolysis?

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Enzymopathy, hemoglobinopathy, membranopathy

Anemia
Anemia

What are the 2 main mutation differences between alpha and beta thalassemia?

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Alpha gene deletion, beta point mutation

Anemia
Anemia

What physical exam / laboratory changes are seen with hereditary spherocytosis?

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Maxillary enlargement, renal tubular acidosis, leg ulcers

Anemia
Anemia

What are the 3 crises that are seen with hereditary spherocytosis?

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Parvovirus B19 aplastic crisis, hyper-hemolytic crisis, megaloblastic crisis

Anemia
Anemia

What is the pathophysiology of hereditary spherocytosis?

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Protein mutations lead to decreased vertical movement, less deformity, less movement in the splenic
venule, leads to hemolysis

Anemia
Anemia

What are the 3 protein mutations of hereditary spherocytosis?

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Band 3, Ankryn, alpha-spectrin

Anemia
Anemia

What is the inheritance of hereditary spherocytosis?

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Autosomal dominant

Anemia
Anemia

Which 2 types of interactions are affected by hereditary spherocytosis versus elliptocytosis?

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Horizontal – elliptocytosis
Vertical – spherocytosis

Anemia
Anemia

How can you treat inherited hemolytic anemias?

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Avoid oxidative stress and perform a splenectomy

Anemia
Anemia

When should you suspect hemoglobinopathy?

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Anemia, autosomal dominant inheritance pattern of anemia, non-spherocyte hemolytic anemia,
hypochromia, bite cells, basophilic stippling, normal hemoglobin electrophoresis

Anemia
Anemia

What tends to send unstable inherited hemoglobinopathies into an acute crisis?

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Oxidative stress

Anemia
Anemia

Which hemoglobin D variant is most severe?

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Hemoglobin SD

Anemia
Anemia

Which hemoglobin variant is seen in India?

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Hemoglobin D

Anemia
Anemia

What is seen on the CBC profile for hemoglobin C?

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Elevated MCHC secondary to water and potassium loss

Anemia
Anemia

What beta chain mutation is seen in hemoglobin C?

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Lysine mutated to glutamate

Anemia
Anemia

Where is hemoglobin C geographically?

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West Africa

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Anemia

What is the third most common hemoglobin?

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Hemoglobin C

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Anemia

What is the second most common hemoglobin mutation?

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Hemoglobin E

Anemia
Anemia

What are the proven changes that can be seen with hydroxyurea in sickle cell anemia?

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Improved overall survival, hemoglobin F, lower pain episodes, lower acute chest, lower need for packed
red blood cell transfusions

Anemia
Anemia

Which red blood cell antigens increase the risk of alloimmunization?

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E, C, Kell

Anemia
Anemia

Which surgical procedures require exchange transfusions versus simple packed red blood cells?

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Orthopedic, hypothermis, obstetric

Anemia
Anemia

What is the problem with having a very rapid increase in hemoglobin and sickle cell anemia?

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Hyperviscosity syndrome when hemoglobin is greater than or equal to 12

Anemia
Anemia

What are some loose indications for exchange transfusion in sickle cell anemia?

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Priapism, pulmonary hypertension, nonhealing leg ulcers

Anemia
Anemia

What are the hemoglobin S concentration and hemoglobin goals for exchange transfusion?

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Hemoglobin S less than or equal to 30%, hemoglobin of 9-10 g/dL

Anemia
Anemia

What are the 5 indications for packed red blood cells in sickle cell anemia?

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Acute spleen sequestration, aplastic crisis, ocular hemorrhage, acute chest with hypoxia, stroke

Anemia
Anemia

How do you manage sickle cell anemia patients while they are pregnant?

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Folic acid and multivitamins

Anemia
Anemia

What is that hemoglobin S concentration target with exchange transfusion?

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Less than or equal to 30%

Anemia
Anemia

How do hydroxyurea and exchange transfusion compare with respect to stroke risk in sickle cell anemia
patients?

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Exchange transfusion is superior

Anemia
Anemia

What feature on transcranial ultrasound is indicative of stroke in sickle cell patients?

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High transcranial velocity

Anemia
Anemia

Which sickle cell disease has the highest risk for stroke?

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Sickle cell anemia

Anemia
Anemia

What are the central nervous system complications of sickle cell anemia?

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Stroke, seizure, intracerebral hemorrhage, subdural hemorrhage, cognitive decline

Anemia
Anemia

What 2 interventions can change or minimize the odds of developing long-term complications of acute
chest syndrome?

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Chronic red blood cell transfusions and hydroxyurea

Anemia
Anemia

When should you consider an exchange transfusion for sickle cell anemia with acute chest syndrome?

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Multi-organ failure, clinical deterioration, chest x-ray with a high number of bilateral infiltrates

Anemia
Anemia

What is the correct treatment dose of hydroxyurea for sickle cell anemia patients?

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35 mg per kilogram per day approximately 2000 mg per day

Anemia
Anemia

Which enzyme does hydroxyurea inhibited when used in sickle cell anemia?

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RNA reductase

Anemia
Anemia

What is the mechanism of pulmonary hypertension in sickle cell anemia?

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Hemoglobin release from homolysis leads to nitrous oxide sequestration causing impaired
smooth muscle relaxation

Anemia
Anemia

What is the #1 cause of death in patients with sickle cell anemia as they age?

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Pulmonary hypertension

Anemia
Anemia

Which bacteria most commonly causes osteomyelitis and sickle cell anemia?

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Salmonella

Anemia
Anemia

Which antigen does parvovirus B19 target on red blood cells?

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P antigen

Anemia
Anemia

What is a pathognomonic feature of parvovirus B19 infection on peripheral smear?

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Pronormoblasts

Anemia
Anemia

What is the difference between delayed hemolytic reaction and hyper-hemolysis?

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Delayed – elevated reticulocytes with no hemoglobin A on electrophoresis
Hyper hemolysis – low reticulocytes – notable hemoglobin A on electrophoresis

Anemia
Anemia

What is the treatment for delayed hemolytic transfusion reaction?

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Steroids with or without IVIG

Anemia
Anemia

How many days after a blood transfusion can a delayed hemolytic reaction occur in SSA patients?

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5-10 days

Anemia
Anemia

What is one way to test for delayed hemolytic transfusion reaction?

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Hemoglobin electrophoresis–normal hemoglobin A detected

Anemia
Anemia

What can worsen kidney problems in sickle cell anemia patients?

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NSAIDs, UTIs, underlying diabetes

Anemia
Anemia Part 3

what is the standard conditioning regimen for matched sibling transplant in aplastic anemia?

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Cyclophosphamide and fludarabine with or without ATG

Anemia Part 3
Anemia

What defines very severe aplastic anemia?

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ANC < 200

Anemia
Anemia

When would you consider packed red blood cell infusions for acute chest syndrome ?

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worsening anemia and hypoxia

Anemia
Anemia

What is the main caution when using fluids for resuscitation in the management of sickle cell acute chest ?

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Pulmonary edema

Anemia
Anemia

What are the most common cause of acute chest syndrome?

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Infection and fat emboli

Anemia
Anemia

What can lower the pneumonia incidence of acute chest syndrome while sickle cell anemia patients are
hospitalized for pain crisis?

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Incentive spirometry

Anemia
Anemia

Which opiate should not be given and why for sickle cell anemia?

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Meperidine causes seizures

Anemia
Anemia

What should management of sickle cell vasoo-cclusive pain crisis always comprise ?

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Hydration

Anemia
Anemia

At what age should retinal examinations start for sickle cell anemia patients?

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10 years old

Anemia
Anemia

What vaccine is required at age 5 for children with sickle cell anemia ?

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Pneumovax booster

Anemia
Anemia

What are the splenic complications of sickle cell anemia?

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Splenomegaly, splenic infarct, hemolytic anemia anemia, sequestration, involution

Anemia
Anemia

Which 2 bacteria cause osteomyelitis in sickle cell anemia patients?

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Staphylococci and Salmonella

Anemia
Anemia

What bacteria commonly causes sepsis in sickle cell anemia children ?

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Strep pneumonia

Anemia
Anemia

What osteopathic pathologies can be seen in patients with sickle cell anemia ?

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Long bone osteomyelitis, avascular necrosis, Dactylitis

Anemia
Anemia

What are some acute causes of hemolytic crisis in sickle cell anemia?

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Parvovirus B19, splenic sequestration, hepatic sequestration, G6PD deficiency flare, autoimmune
hemolytic anemia flare

Anemia
Anemia

Which metabolite is increased as a compensatory mechanism for autoimmune hemolytic anemia?

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2,3-BPG

Anemia
Anemia

What is the hemoglobin electrophoresis ratio difference between SB+ and AS?

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A/S 60/40 while B+/S = 15/85

Anemia
Anemia

Which laboratory method is used for sickle cell diagnosis?

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High pressure liquid chromatography

Anemia
Anemia

What CBC features can distinguish sickle cell versus sickle beta(0)?

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MCV in SS normal while in SB(0) its low

Anemia
Anemia

What causes a vaso-occlusive crisis in sickle cell anemia?

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Sickle cells + neutrophils + platelets clog up the vasculature

Anemia
Anemia

Which 2 types of sickle cell disease are the most severe?

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SS/SB(0)

Anemia
Anemia

What is the A/S ratio of hemoglobin electrophoresis for SS trait?

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A/S = 60/40

Anemia
Anemia

What are the 6 major complications of sickle cell trait?

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Death from fever and dehydration, hematuria, pyelonephritis during pregnancy, spleen infarct at high
altitude, kidney papillary necrosis, kidney medullary carcinoma

Anemia
Anemia

What are the 7 different types of sickle cell disease?

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SS, SC, SB(0) , SB+, SO (Arab), SD (Punjab), SE

Anemia
Anemia

How do you manage alpha thalassemia major?

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Splenectomy and chronic transfusions

Anemia
Anemia

How do you manage intrauterine _ _ / _ _ Thalassemia?

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Intrauterine packed red blood cell and postnatal allogeneic stem cell transplant

Anemia
Anemia

Which 2 forms of hemoglobin will be predominant in Hydrops Fetalis?

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Delta 2 beta-2 and Zeta -2 Gamma 2

Anemia
Anemia

When would you see hemoglobin H?

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_ _ / a a

Anemia
Anemia

Which combination of hemoglobinopathy makes _a/ _ _worse?

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Combination of hemoglobin H and constant spring

Anemia
Anemia

What is the severity of hemoglobin constant Spring?

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Moderate anemia

Anemia
Anemia

What protein does the ATRX gene translate?

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Chromagen remodeling factor

Anemia
Anemia

What disease does ATRX gene deletion lead to?

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ATMDS – alpha thalassemia/ MDS syndrome

Anemia
Anemia

Which 2 tetramers will form and which will precipitate out with _ _ / _ a thalassemia ?

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Gamma 4 tetramers – Barts
Beta 4 – hemoglobin H – precipitates out

Anemia
Anemia

What does the Greek symbol Tao represent when next to the alpha symbol for thalassemia?

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Point mutation – seen in hemoglobin constant Spring

Anemia
Anemia

What is the difference between the alpha thalassemia mutation seen in those of Asian versus African ancestry ?

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Asians – cis _ _/aa

AA – trans _a/_a

Anemia
Anemia

Which ethnic group in the United States has one third _a/aa in their gene pool?

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African-American

Anemia
Anemia

Which protein promotor loss leads to alpha thalassemia _ _/ _ _ ?

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HS40

Anemia
Anemia

Which geographic area is NOT shared between the alpha and beta thalassemia gene pools ?

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The Middle East – no alpha thalassemia present

Anemia
Anemia

At what age should you consider splenectomy for beta thalassemia?

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5 years of age

Anemia
Anemia

What are the hemoglobin and ferritin goals for beta thalassemia management ?

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Hemoglobin 9.5 g/dL
Chelation should start once the ferritin is above 1,000 ng/mL

Anemia
Anemia

What are thetypical MCH, MCV and Hgb values for beta-thalassemia minor ?

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MCV less than 70fL, hemoglobin between 9 and 11 g/dL, Hgb A2 > 3.5%, low MCH

Anemia
Anemia

How much hemoglobin A should be seen in a Beta-0____ patient?

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0

Anemia
Anemia

Which 2 other types of hemoglobin increase on hgb-electropheresis with beta thalassemia?

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Hemoglobin F and hemoglobin A2

Anemia
Anemia

What are the consequences of isolated epsilon, gamma, delta, beta thalassemia?

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None

Anemia
Anemia

What is the most serious complication of splenectomy for beta thalassemia?

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Deep venous thrombosis and central nervous system thrombosis

Anemia
Anemia

What central nervous system complication can occur with beta thalassemia?

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Thromboses status post splenectomy

Anemia
Anemia

What infection is common with iron chelation therapy with beta thalassemia?

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Yersinia enterocolitis

Anemia
Anemia

What bone changes are seen with Cooley anemia?

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Osteopenia, thin cortex, thick marrow, fractures

Anemia
Anemia

What 4 genetic changes affect the severity of beta thalassemia?

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Severity of beta gene mutation, plus or minus alpha defects, plus or minus gamma defects, plus or
minus sickle cell genes

Anemia
Anemia

What red blood cell changes are seen with beta thalassemia major?

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Alpha – 4 tetramers precipitation

Anemia
Anemia Part 4

What biliary complications are seen with sickle cell anemia?

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Gallstones

Anemia Part 4
Anemia

What are the 5 indications for packed red blood cell use in sickle cell anemia patients?

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Acute chest, symptomatic anemia, acute stroke, surgical preparation, multiorgan failure

Anemia
Anemia

Which opiates are preferred for acute pain crisis in sickle cell anemia?

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Short acting opiates, avoid NSAIDs

Anemia
Anemia

At what age do ophthalmology retinal exams begin in sickle cell anemia?

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After age 10

Anemia
Anemia

How do you screen for stroke in sickle cell anemia kids?

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Transcranial Doppler, starting at age 2, until age 16

Anemia
Anemia

What ophthalmology pathology do sickle cell anemia patients develop ?

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Proliferative retinopathy

Anemia
Anemia

Which vitamins and minerals do sickle cell anemia patients need to supplement with?

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Folic acid, calcium, vitamin D

Anemia
Anemia

Which prophylactic antibiotics do sickle cell anemia patients need as children?

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Penicillin until age 5

Anemia
Anemia

Which vaccines do sickle cell anemia patients need?

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Encapsulated bacteria and hepatitis B

Anemia
Anemia

How many weeks should you wait in myelodysplastic syndrome until a response to ESA is seen?

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12 weeks

Anemia
Anemia

What criteria can be used to distinguish a good responder for myelodysplastic syndrome to use ESA?

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Less than 2 units of packed red blood cells every month, IP SS–1 intermediate low-risk, EPO less than 500, less than 10% blasts in bone marrow

Anemia
Anemia

Which antibody mediates drug-induced AIHA?

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IgG- and C3+

Anemia
Anemia

What is the antibody difference between cold agglutinin disease and PCH?

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CA = IgM
PCH- IgG

Anemia
Anemia

If patients don’t have a response to warm packed red blood cells with cold AIHA what is a treatment option?

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Plasmapheresis

Anemia
Anemia

Which antibody mediates Cold AIHA?

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IgM

Anemia
Anemia

What is the treatment for cold AIHA?

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Chemo-immunotherapy such as FCR

Anemia
Anemia

What is the difference between cold and warm autoimmune hemolytic anemia with respect to mechanism site?

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Warm – spleen
Cold – liver

Anemia
Anemia

What are the hemoglobin, ferritin, transferrin saturation goals for EPO use?

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Hemoglobin < 11Ferritin > 100
Transfer greater than 20%

Anemia
Anemia

What is the treatment frequency for EPO pre- and post-hemodialysis?

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Pre-hemodialysis every 7 days
Post hemodialysis every 21 days

Anemia
Anemia

What minimal titer is required to develop clinically significant hemolytic anemia?

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>1:64

Anemia
Anemia

What is the difference between EPO-alpha, EPO-beta, and darbopoetin ?

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Post translational glycosylation

Anemia
Anemia

Which signaling pathway is used by the EPO receptor?

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JAK2/STAT

Anemia
Anemia

What is the surface immunoglobulin pattern for cold agglutinin disease?

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IgG- and C3+

Anemia
Anemia

What % of patients with autoimmune hemolytic anemia respond to steroids?

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70%

Anemia
Anemia

What happens when you use G-CSF concurrent with chemotherapy and radiotherapy?

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Severe constitutional symptoms

Anemia
Anemia

Which group of congenital anemias should not get ESA growth factor?

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Sickle cell anemia from pain crisis

Anemia
Anemia

What is the most common warm autoimmune hemolytic anemia pattern?

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IgG++ and C3++

Anemia
Anemia

What non-leukemia indications are there for growth factor use?

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Cyclic neutropenia, congenital neutropenia, bone marrow transplant, myelodysplastic syndrome

Anemia
Anemia

What is the most concerning lifetime risk for patient with congenital neutropenia?

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30% risk of AML

Anemia
Anemia

What would a bone marrow biopsy show in a patient with Copper deficiency?

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Hypocellular marrow with dyserythropoesis

Anemia
Anemia

Which Copper salt is used in the treatment of Copper deficiency?

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Copper chloride

Anemia
Anemia

Which mineral deficiencies should always be considered for bypass surgery patients that have refractory anemias?

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Copper and Zinc

Anemia
Anemia

Which leukemia has no change in overall survival with growth factor?

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ALL – meta-analysis shows no change in overall survival

Anemia
Anemia

What portion of acute myeloid leukemia therapy does G-CSF have a proven role in?

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Consolidation chemotherapy only
No role for induction chemotherapy

Anemia
Anemia

What is the only FDA indication for sargramostim?

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Induction chemotherapy for AML for those older than 55

Anemia
Anemia

What is the EPO level at which ESA agents are most effective?

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EPO

Anemia
Anemia

What kidney problems are sickle cell trait patients at high risk of developing?

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Renal medullary cancer

Anemia
Anemia

What is the biggest concern for aplastic crisis in sickle cell anemia patients?

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Encapsulated bacterial infections

Anemia
Anemia

What is the biggest concern with meperidine for sickle cell anemia?

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Seizure risk increase

Anemia
Anemia

What is the recommendation for pregnant patients with sickle cell anemia?

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No specific transfusion recommendations

Anemia
Anemia

What are the 6 indications in the acute setting for simple packed red blood cell transfusions for sickle cell anemia patients?

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Symptomatic anemia, aplastic crisis, spleen or liver sequestration, acute chest syndrome, multiorgan failure, preoperative transfusion for major surgery

Anemia
Anemia

What is an ideal preoperative hgb for sickle cell anemia patients?

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10 g/dL

Anemia
Anemia

Which mammal is most commonly used as a source for generating ATG ?

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Horse

Anemia
Anemia

What is ATG given in combination with for young patients with severe aplastic anemia?

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Cyclosporine

Anemia
Anemia

Which clone can sometimes be seen in patients with newly diagnosed aplastic anemia?

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PNH

Anemia
Anemia

What serum trough is the target for aplastic anemia when being treated with cyclosporine?

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200-400 ng/mL

Anemia
Anemia

When would you consider the addition of a TPO mimetic in aplastic anemia?

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No response seen with first line ATG/CsA

Anemia
Anemia

What defines non-severe aplastic anemia?

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ANC >500

Anemia
Anemia

How many days after first-line treatment with ATG/CsA is a failure announced ?

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120 days

Anemia
Anemia

After what age is there a significant decline in transplant associated survival for aplastic anemia ?

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40 years old

Anemia
Anemia Part 5

In which atypical anatomic sites can paroxysmal nocturnal hemoglobinuria patients develop deep venous thrombosis?

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Hepatic vein, cerebral vein, venous sinuses, abdominal veins

Anemia Part 5
Anemia

What is the relationship between paroxysmal nocturnal hemoglobinuria and malignant hematology?

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20% of myelodysplastic syndrome patients and 20% of aplastic anemia patients have a paroxysmal nocturnal hemoglobinuria clone

Anemia
Anemia

What are other clinical manifestations for paroxysmal nocturnal hemoglobinuria besides hemoglobinuria?

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Esophageal spasms, deep venous thrombosis, severe fatigue, pulmonary hypertension, erectile dysfunction

Anemia
Anemia

What are the cell surface targets of FLAER diagnostic flow cytometry for paroxysmal nocturnal hemoglobinuria?

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CD55

Anemia
Anemia

Which cells are targeted on flow cytometry for paroxysmal nocturnal hemoglobinuria diagnosis?

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Monocytes and neutrophils

Anemia
Anemia

How can you increase the hemoglobin in the sickle cell anemia if patient needs to have surgery?

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Simple transfusion and not an exchange transfusion

Anemia
Anemia

Which 2 enzymes are affected in paroxysmal nocturnal hemoglobinuria that leads to deep venous thrombosis?

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Urokinase and TFPI – decreased fibrinolysis

Anemia
Anemia

Where does the hemolysis in paroxysmal nocturnal hemoglobinuria occur?

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Intravascular hemolysis

Anemia
Anemia

Which gene is defective in paroxysmal nocturnal hemoglobinuria?

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PIG-A

Anemia
Anemia

What are 4th line treatment options for warm autoimmune hemolytic anemia?

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Cyclosporine, azathioprine, MMF, cyclophosphamide

Anemia
Anemia

What should be offered if there is no improvement in autoimmune hemolytic anemia with steroids?

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Splenectomy

Anemia
Anemia

Which ITP treatment does not work in warm autoimmune hemolytic anemia?

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IVIG

Anemia
Anemia

How should packed red blood cells be transfused when there is severe autoimmune hemolytic anemia?

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Very slowly

Anemia
Anemia

How can you increase the hemoglobin in the sickle cell anemia if patient needs to have surgery?

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Simple transfusion and not an exchange transfusion

Anemia
Anemia

Which treatments work in ITP, warm AIHA, cold AIHA?

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Rituximab

Anemia
Anemia

What are the causes of IgG +/- C3 + DAT?

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IgG + warm AIHA , drug
IgG+/C3+ – Paroxysmal cold, Drug AIHA, Warm AIHA, Cold agglutinin Disease
C3+ – warm AIHA and Drug

Anemia
Anemia

Which 2 treatments that are used for warm and AIHA do not have a role in cold AIHA ?

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Splenectomy and steroids

Anemia
Anemia

What is the difference in severity between Hapten and ternary/Immune Complex AIHA?

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Hapten- mild
Ternary – life-threatening

Anemia
Anemia

If there is a high clinical suspicion for autoimmune hemolytic anemia but negative Coombs, what test can be performed?

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ELISA, radiolabeled anti-Ig, and IgA antibody

Anemia
Anemia

What is the time course for Hapten/drug adsorption mediated AIHA?

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7-10 days

Anemia
Anemia

What is the most severe example of autoantibody mediated autoimmune hemolytic anemia?

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Fludarabine – severe CLL – life-threatening AIHA

Anemia
Anemia

What is the difference in mechanism seen with Hapten versus immune complex mediated autoimmune hemolytic anemia?

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Hapten / drug adsorption = IgG mediated spleen clearance
Ternary/Immune Complex = complement mediated intravascular

Anemia
Anemia

What is the most common cause of drug-induced autoimmune hemolytic anemia?

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Second and third generation cephalosporins

Anemia
Anemia

What is an important difference with cold autoimmune hemolytic anemia and syphilis in children?

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Donath – Lansteiner = IgG that fixes complement

Anemia
Anemia

Which 2 infections are associated with cold autoimmune hemolytic anemia?

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EBV and mycoplasma pneumonia

Anemia
Anemia

Which red blood cell carbohydrate on the surface does IgM bind to in cold agglutinin disease?

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I or i

Anemia
Anemia

What is the primary mechanism with respect to location and destruction in warm versus cold autoimmune hemolytic anemia ?

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Cold is IgM mediated causing breast blood cell agglutination in the liver
Warm is IgG mediated with RBC coated by IgG, destruction in the spleen

Anemia
Anemia

With conditions are warm and cold autoimmune hemolytic anemia associated with?

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SLE and autoimmune disease.

Anemia
Anemia

What is cold autoimmune hemolytic anemia associated with?

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Cold agglutinin disease, infectious mono, mycoplasma pneumoniae, B-cell lymphoproliferative disorder, paroxysmal cold hemoglobinuria secondary to a tertiary syphilis secondary to Donath Lansteiner Vegetations

Anemia
Anemia

What are warm autoimmune hemolytic anemia usually felt secondary to?

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Lymphoma, rheumatoid disease, solid tumors, autoimmune disease, drugs

Anemia
Anemia

What are the 3 different types of drug mediated hemolytic anemia?

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New drug – adsorption onto red blood cell membrane
Haptens with cold exposure
Immune complex – neoantigen created by red blood cell surface antigen/drug

Anemia
Anemia

What percentage of autoimmune hemolytic anemia is cold?

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15%

Anemia
Anemia

When should you check a G6PD level to confirm a diagnosis?

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After the hemolytic event has finished – 4-6 weeks later

Anemia
Anemia

What is seen on the peripheral smear for G6PD?

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Hines bodies, ghost cells (eccentrocytes)

Anemia
Anemia

What are 3 different types of G6PD variants and their respective levels of severity?

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A=normal variant and no symptoms
B=mild
Mediterranean = severe

Anemia
Anemia

Which molecule mediates esophageal spasms when there is free-floating hemoglobin?

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Nitrous oxide

Anemia
Anemia

What are the 3 main post- embryonic hemoglobin subtypes?

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Alpha-2 beta-2 is hemoglobin A
Alpha-2 delta-2 is hemoglobin A2
Alpha-2 gamma -2 is hemoglobin F

Anemia
Anemia

What are the other hemoglobin genes?

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Delta, Exelon, gamma, Zeta

Anemia
Anemia

Which chromosomes have the alpha and beta genes for hemoglobin?

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Chromosome 11 has Beta, Chromosome 16 has the alpha gene.

Anemia
Anemia

Which 2 chromosomes carry the hemoglobin genes?

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11 and 16

Anemia
Anemia

What are 3 classification schemes for hemolytic anemia?

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Intravascular versus extravascular, hereditary versus sporadic, intrinsic versus extrinsic

Anemia
Anemia

When can you stop penicillin prophylaxis in sickle cell anemia?

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Stop at age 5 and they should get the pneumonia vaccine

Anemia
Anemia

What 2 urine changes are seen with hemolytic anemia?

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Hemosiderin as well as urine urobilinogen

Anemia
Anemia

What helps leg ulcers besides supportive care in sickle cell anemia?

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Hydroxyurea and packed red blood cells

Anemia
Anemia

What are leg ulcers are associated with in sickle cell anemia?

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50% DVT as well as pulmonary hypertension

Anemia
Anemia

What dermatologic problem is seen in sickle cell anemia?

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Leg ulcers

Anemia
Anemia

Which vaccine do sickle cell anemia patients require?

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Inactivated starting at age 6 months

Anemia
Anemia

When should pneumonia vaccine start for sickle cell anemia patients?

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PVC-13 (conjugate) ASAP and PPSV23 (polysaccharide) – after age 2

Anemia
Anemia

What is the Greek acronym for fetal hemoglobin?

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Alpha-2 delta 2

Anemia
Anemia

How does hydroxyurea decreas pain crises in sickle cell anemia?

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Lower polymerization by increasing fetal hemoglobin

Anemia
Anemia Part 6

Which red blood cell surface antigens are cold agglutinins developing antibodies to?

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I (upper case) antigen (NOT i, lower case)

Anemia Part 6
Anemia

What are common triggers for G6PD deficiency flares?

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Sepsis, liver injury, drugs, DKA

Anemia
Anemia

What is the most common Hexose-Monophosphate shunt abnormality?

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G6PD deficiency

Anemia
Anemia

Which other enzymes from the glycolytic pathway can be affected in enzymatic anemia?

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Pyruvate kinase, Isomerase, hexokinase, phosphokinase kinase

Anemia
Anemia

How can you distinguish spherocytosis from pyruvate kinase deficiency?

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Normal osmotic fragility test in pyruvate kinase versus hereditary spherocytosis

Anemia
Anemia

What is the most common enzyme abnormality in enzymatic anemia?

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Pyruvate kinase

Anemia
Anemia

Which 2 pathways provide energy for a red blood cell?

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Glycolysis and Hexose-monophosphate shunt

Anemia
Anemia

What is the mechanism of clearance for babesiosis?

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Splenic clearance

Anemia
Anemia

What is a unique peripheral smear feature for Babesiosis?

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Rings

Anemia
Anemia

Which insect transmits bartonella?

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Sandfly

Anemia
Anemia

What occurs in the acute and chronic phase of Bartonella?

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Acute hemolysis
Chronic cutaneous granulomatous disease

Anemia
Anemia

What is the incubation period for babesiosis?

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1-4 weeks

Anemia
Anemia

What is the location of hemolysis for babesiosis?

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Intravascular hemolysis

Anemia
Anemia

What can make babesiosis more severe?

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Asplenia

Anemia
Anemia

Which ticks cause babesiosis?

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Ixodes tick

Anemia
Anemia

How do you treat the anemia of malaria?

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Quinine and chloroquine

Anemia
Anemia

What 3 enzmyopathies should be considered with respect to ethnicity and geography?

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G6PD deficiency, thalassemia, hemoglobinopathy

Anemia
Anemia

What stages of red blood cell maturation do the 3 different malaria parasites infect?

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Reticulocytes P vivax and P ovale
RBC – P malariae
All stages – P falciparum

Anemia
Anemia

What is the #1 cause of hemolytic anemia worldwide?

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Malaria

Anemia
Anemia

Which 2 infections can cause cold agglutinin disease?

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Epstein-Barr virus can cause mononucleosis and mycoplasma pneumoniae

Anemia
Anemia

What are two mechanisms of destruction of cold autoimmune hemolytic anemia?

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Macrophage opsonization and complement mediated hemolysis

Anemia
Anemia

Which viruses besides Epstein-Barr virus can cause hemolysis?

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CMV, herpes virus, influenza, rubella, varicella

Anemia
Anemia

What are the treatment options for cold agglutinin disease if there is no self-resolution?

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Plasmapheresis

Anemia
Anemia

With temperature threshold is needed to see cold agglutinin disease?

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32°C

Anemia
Anemia

Which gram-positive and gram-negative bacteria can cause hemolytic anemia?

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Staph, strep, enterococcus

Anemia
Anemia

What are the severe consequences of C. difficile colitis and hemolytic anemia?

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Liver and kidney failure

Anemia
Anemia

Which enzyme from C. difficile can cause severe hemolytic anemia?

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Phospholipase C

Anemia
Anemia

Which common hospital-acquired infections can cause a hemolytic anemia?

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C. difficile

Anemia
Anemia

Which parasites can cause hemolytic anemia?

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Babesiosis, Bartonella, malaria

Anemia
Anemia

With hepatitis C treatment can cause hemolytic anemia?

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Ribavarin

Anemia
Anemia

Which mineral can cause hemolytic anemia?

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Copper

Anemia
Anemia

Which head and neck cancer treatment modality can lead to hemolytic anemia?

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Radiotherapy

Anemia
Anemia

Which syndrome in kids will have microangiopathic hemolytic anemia and skin changes?

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Kassabach-Merrit syndrome

Anemia
Anemia

What gestation cutoff can distinguish TTP and HUS from acute fatty liver of pregnancy and HELLP syndrome?

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Less than 20 weeks for TTP and HUS

Anemia
Anemia

Which solid tumors can cause disseminated intravascular coagulation?

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Mucin producing tumors

Anemia
Anemia

Which drugs can cause a microangiopathic hemolytic anemia picture?

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Gemcitabine, mitomycin, cyclosporine, Taxcrolimus, ticlodipine, clopidegrel

Anemia
Anemia

With can look like hemolytic uremic syndrome and TTP on a peripheral smear?

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Malignant hypertension

Anemia
Anemia

What 6 diseases are on the MAHA Spectrum?

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HELLP, AFLP, DIC, TTP, aHUS, HUS

Anemia
Anemia

What cardiovascular causes can also produce hemolysis?

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Congestive heart failure, cordae tendinease rupture, aortic aneurysm, balloon pump, cardiac assist devices

Anemia
Anemia

What is the treatment for mechanical valve associated hemolysis?

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Valve replacement

Anemia
Anemia

Which animal can cause hemolytic anemia?

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Snake bites from vipers

Anemia
Anemia

What are non-microvascular causes of fragmentation hemolysis?

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March hemoglobin urea and mechanical valve destruction

Anemia
Anemia

Which staining is positive when there is fragmentation hemolysis?

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Hemosiderin

Anemia
Anemia

What are the common causes of death for patients with paroxysmal nocturnal hemoglobinuria ?

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AML, thrombosis, pancytopenia

Anemia
Anemia

Which vaccine is required for patients undergoing treatment for paroxysmal nocturnal hemoglobinuria with eculizumab?

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Neisseria meningitidis

Anemia
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