Heme

Question 1

Along with homocysteine, what levels can be elevated with vitamin B12 deficiency?

Answer 1

Methylmalonic acid levels

Question 2

Besides elevated MCV, what else can be elevated with folate deficiency?

Answer 2

Homocysteine levels

Question 3

Blister cells or bite cells on peripheral smear are most often seen with what condition?

Answer 3

Glucose 6 phosphate dehydrogenase deficiency (G6PD)

Question 4

Bleeding disorder with GpIb deficiency?

Answer 4

Bernard-Soulier syndrome (defect in platelet adhesion to von Willebrand factor)

Question 5

Name the four main causes of microcytic anemia.

Answer 5

Thalassemia, Iron deficiency, Anemia of chronic disease, Sideroblastic anemia (mnemonic TICS)

Question 6

What are the most common vitamin K dependent clotting factors?

Answer 6

Factors II, VII, IX, and X

Question 7

Patient is found to have a Pelger-Huet anomaly and hyposegmented neutrophils. What condition does this patient likely have?

Answer 7

Myelodysplastic syndrome

Question 8

What condition is often associated with hypersegmented neutrophils?

Answer 8

Vitamin B12 deficiency

Question 9

What hematologic condition is often associated with restless leg syndrome?

Answer 9

Iron deficiency anemia

Question 10

What hematological condition is caused by a membrane defect of spectrin deficiency?

Answer 10

Hereditary spherocytosis

Question 11

What is the earliest lab value to increase after repleting iron in patients with iron deficiency anemia?

Answer 11

Reticulocyte count

Question 12

Rapid fire assoc:Stacks of RBCs

Answer 12

Rouleaux formation (high ESR, multiple myeloma)

Question 13

Anaphylaxis following blood transfusion Dx?

Answer 13

IgA deficiency

Question 14

Autosplenectomy (fibrosis and shrinkage) associated w/what dz?

Answer 14

Sickle cell disease (hemoglobin SS dz)

Question 15

Rapid fire txt: Heparin reversal

Answer 15

Protamine sulfate

Question 16

Rapid fire assoc:Monoclonal antibody spike. 3 things.

Answer 16

-Multiple myeloma (usually IgG or IgA)
-Monoclonal gammopathy of undetermined significance (MGUS consequence of aging)
Waldenström (M protein= IgM) macroglobulinemia
-Primary amyloidosis

Question 17

dabigatran bleeding reversal agent?

Answer 17

Idracizumab or prothrombin complex concentrate

Question 18

Painful blue fingers/toes, hemolytic anemia

Answer 18

Cold agglutination disease (autoimmune hemolytic anemia caused by Mycoplasma pneumoniae, infectious mononucleosis, CLL)

Question 19

Rapid fire assoc: Hypochromic, microcytic anemia

Answer 19

Iron deficiency anemia, lead poisoning, thalassemia (fetal hemoglobin sometimes present)

Question 20

Mucosal bleeding and prolonged bleeding time

Answer 20

Glanzmann thrombasthenia (defect in platelet aggregation due to lack of GpIIb/IIIa)

Question 21

Rapid fire assoc:Basophilic nuclear remnants in RBCs

Answer 21

Howell-Jolly bodies (due to splenectomy or nonfunctional spleen)

Question 22

Rapid fire assoc:Hair on end” (“Crew cut”) appearance on x-ray

Answer 22

β-thalassemia, sickle cell disease (marrow expansion)

Question 23

Rapid fire assoc: basophilic stippling on RBCs

Answer 23

Lead poisoning or sideroblastic anemia

Question 24

Rapid fire assoc: Antiplatelet antibodies

Answer 24

Idiopathic thrombocytopenic purpura

Question 25

What is the simplest test to differentiate anemia of chronic disease from iron deficiency?

Answer 25

Total iron-binding capacity (TIBC)

Question 26

What mutation is often associated with polycythemia vera?

Answer 26

JAK2 mutation

Question 27

Athlete with polycythemia Dx?

Answer 27

Secondary to erythropoietin injection (blood doping)

Question 28

Which antibody is associated with cold agglutinin hemolytic anemia?

Answer 28

IgM

Question 29

If mixing study corrects PT or PTT, what would this indicate?

Answer 29

Indicates a factor deficiency.

Question 30

Myelodysplasia w/5q and w/out 5q txt?

Answer 30

Lenalidomide, azacitidine

Question 31

What are the 2 most common causes of spherocytosis?

Answer 31

Auto immune hemolytic anaemia & hereditary spherocytosis

Question 32

How do you differentiate

Auto immune hemolytic anaemia & hereditary spherocytosis?

Answer 32

Direct anti globulin test (coombs). DAT is negative in HS patients

Question 33

How do you confirm the diagnosis of hereditary spherocytosis?

Answer 33

Eosin 5 maleimide binding (flow cytometry) or acidified glycerol lysis test

Question 34

How do you treat hereditary spherocytosis?

Answer 34

Rbc transfusion for symptomatic anemia, folic acid supplementation, splenectomy for severe cases

Question 35

What are the 3 main diseases that patients with polycythemia Vera are at risk of developing?

Answer 35

Thrombotic events, post polycythemia myelofibrosis, acute myeloid leukaemia/Myelodysplastic syndrome

Question 36

Abdominal pain, constipation, dark urine, hyponatraemia, peripheral neuropathy, neuropsychiatric manifestations suggest what diagnosis?

Answer 36

Acute intermittent porphyria

Question 37

What is the dx test and treatment for Acute intermittent porphyria?

Answer 37

Elevated urinary porphobilinogen (PBG) during attack. Glucose loading and intravenous hemin

Question 38

What is the most common long term adverse effect of external beam radiation for prostate cancer?

Answer 38

Sexual dysfunction