Hematology

Question 1

What factors are associated with the intrinsic pathway and result in elevated pTT?

Answer 1

factor 8, 9, 11

Question 2

What is the most likely diagnosis for patient with bleeding and abnormally large platelets (increased mean platelet volume)?

Answer 2

Bernard Soulier syndrome

due to glycoprotein 1B deficiency

Question 3

What is the most likely diagnosis for patient with microcytic anemia not responsive to iron therapy and increased HbA2?

Answer 3

Beta thalassemia trait

Question 4

What is the most likely complication of a large hemangioma that is increasing in size?

Answer 4

thrombocytopenia

known as Kasabach-Merritt phenomena or hemangioma thrombocytopenia syndrome

Question 5

What is the most likely diagnosis for patient with anemia, hepatosplenomegaly, elevated direct bilirubin, gallstones, small hyperchromic perfectly round cells without central pallor and elevated MCHC (mean corpuscular Hg concentration) and jaundice?

Answer 5

Hereditary Spherocytosis

Question 6

What is the diagnostic test of choice for Hereditary Spherocytosis?

Answer 6

Osmotic fragility test

Question 7

What is the most likely complication to occur in a Sickle Cell disease patient less than 2 years old?

Answer 7

Dactylitis

pain symmetrical swelling of hands and feet

Question 8

What is post circumcision bleeding especially with elevated pTT?

Answer 8

Hemophilia (likely factor 8, 9, 11 deficiency)

Question 9

What is the most common cause of pancytopenia in toddlers?

Answer 9

Acute leukemia

dx: bone marrow aspirate

Question 10

What is the most likely diagnosis for patient presenting with thrombocytopenia followed by anemia and neutropenia with hypocellular bone marrow with fatty infiltration, along with associated cutaneous hyperpigmentation, GI atresia, radial abnormalities, short stature and risk of acute myeloid leukemia?

Answer 10

Fanconi anemia

Question 11

When should iron supplementation begin if not present in formula?

Answer 11

4-6 months old (iron stores deplete at this age if not in milk)

Question 12

What is the most likely diagnosis for African American/ Mediterranean male pt with hemolytic anemia in response to taking primaquine/ fava beans/ vitamin K analogs/ aspirin with bite/ blister cells on peripheral smear?

Answer 12

G6PD deficiency

Question 13

What is the most likely etiology of transient aplastic anemia (anemia with low reticulocyte count) in sickle cell disease patient?

Answer 13

Parvovirus B19 (fifth disease)

Question 14

What is the Mentzer index and what is it used for?

Answer 14

MCV/ RBC
iron deficiency- increased
thalassemia- decreased

Question 15

What is the most likely diagnosis associated with short webbed neck, macrocytic anemia, decreased retic count, decreased RBC precursors, light hair, snubbed nose and other congenital abnormalities?

Answer 15

Diamond Blackfan anemia (Congenital hypoplastic anemia)

Question 16

What is the most likely diagnosis for patient who develops hemolytic anemia (schistocytes), thrombocytopenia, decreased PT, decreased PTT, decreased fibrinogen and elevated D-dimer in response to trauma/ sepsis/ malignancy?

Answer 16

DIC (disseminated intravascular coagulation)

Question 17

What is the best treatment for ITP (idiopathic thrombocytopenic purpura)?

Answer 17

IVIG or anti-Rh (D) Ig

if active bleeding; no tx needed if no active bleeding

Question 18

What is the most likely diagnosis for patient with decreased UOP, hyperbilirubinemia, elevated LDH, anemia, thrombocytopenia?

Answer 18

Thrombotic thrombocytopenic purpura (TTP)

tx: plasmapheresis and steroids

Question 19

What is the pathophysiology related to thrombotic thombocytopenic purpura (TTP)?

Answer 19

ADAMTS13 antibodies

Question 20

What is the most likely diagnosis for patient with tetralogy of fallot, decreased megakaryocytes and bilateral radial aplasia?

Answer 20

Thrombocytopenic absent radius (TAR)

Question 21

What is the most likely diagnosis associated with Heinz bodies (intracellular inclusions in RBCs)?

Answer 21

G6PD deficiency

Question 22

What do white blood cells with bilobed nuclei and reddish granules intracellular on blood smear suggest?

Answer 22

Eosinophilia

Question 23

What are two complications of sickle cell trait?

Answer 23

1. Hyposthenuria (inability to concentrate urine)

2. renal papillary necrosis (with gross hematuria)

Question 24

What is the most likely diagnosis for patient under 2 year old with persistent neutropenia and bone marrow showing granulocytopenia with normal trillenage precursors?

Answer 24

Chronic benign neutropenia

due to autoantibodies to granulocytes

Question 25

What diagnosis is associated with patient having sclerosis of long bone showing “bone within bone appearance” reflecting old cortex within new cortex?

Answer 25

Sickle Cell Disease

Question 26

What is kolionychia (spooning of fingernails or toenails) associated with?

Answer 26

iron deficiency anemia

Question 27

What three clotting factors are not synthesized in the liver?

Answer 27

1. Factor 8
2. Tissu factor
3. vonWillebrand factor

Question 28

What is the most likely diagnosis for patient presenting with thrombocytopenia, small platelets and history of eczema?

Answer 28

Wiskott-Aldrich syndrome

tx: stem cell transplant

Question 29

What is the most likely diagnosis for patient presenting with neutropenia, infection with Clostridium septicum, aphthous ulcers, stomatitis, and recurrent bacterial infection?

Answer 29

Cyclic neutropenia

dx: Neutropenia on CBC 2x/ week for 6 weeks at least

Question 30

What is a possible side effect of the use of hydroxyurea in sickle cell patients?

Answer 30

leukocyte suppression (decreased WBCs)

Question 31

What is the most likely diagnosis for patient with recurrent infections, failure to thrive, pancreatic exocrine insufficiency (malabsorption), neutropenia and normal sweat chloride test?

Answer 31

Shwachman- Diamond Syndrome

Question 32

What is the most likely diagnosis of patient with acute anemia, icterus, jaundice, splenomegaly, increased LDH, increased retic count, and positive direct Coombs?

Answer 32

Autoimmune hemolytic anemia

Question 33

What is the most dangerous form of neutropenia?

Answer 33

Kostmann syndrome (prolonged neutropenia without reserves)

Question 34

What is the most likely diagnosis with neutropenia, no marrow reserves and does not respond to G-CSF?

Answer 34

Kostmann syndrome

Question 35

What is the most common cause of osteomyelitis in sickle cell disease patient?

Answer 35

non-typhoidal Salmonella

Question 36

What hematologic disorder results in activated protein C resistance (activated protein C unable to function properly)?

Answer 36

Factor 5 Leiden

activated protein C unable to cleave Factor 5

Question 37

What is basophilic stippling on blood smear suggest?

Answer 37

Lead Poisoning

Question 38

Hypersegmented neutrophils are associated with …

Answer 38

Folate deficiency (macrocytic anemia)

Question 39

What is the treatment for acute priaprism (prolonged painful erection) in sickle cell disease patient?

Answer 39

aspiration and irrigation of corpus cavernosum

if IV fluids and analgesic does not resolve

Question 40

Is anemia alone an indication for transfusion in a sickle cell disease patient?

Answer 40

No

Question 41

What is the first abnormality noted in acute liver failure?

Answer 41

prolonged PT (prothrombin time)

Question 42

What is the most likely diagnosis for patient with frontal bossing, flattened nasal bridge, maxillary hyperplasia, hepatosplenomegaly, hypochromic microcytic anemia, target cells and basophilic stippling?

Answer 42

Beta Thalassemia major

Question 43

What clotting factors are associated with Vitamin K (depleted with warfarin treatment)?

Answer 43

Factor 2, Factor 7, Factor 9, Factor 10, protein S, and protein C