Heme- pathoma

Question 1

What is the very first step in primary hemostasis?

Answer 1

TRANSIENT vasoconstriction

Question 2

Two sources of vWF?

Answer 2

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Question 3

Etiology of TTP (Thrombotic Thrombocytopenic Purpura)

Answer 3

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Question 4

TTP/HUS

• bleeding time
• PT
• PTT

Answer 4

• bleeding time
• PT
• PTT

Question 5

Blood smear finding in Bernard- Soulier syndrome

Answer 5

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Question 6

Clinical lab work to distinguish hemophilia A vs. factor 8 inhibitor

Answer 6

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Question 7

What is the most common inherited bleeding disorder? Inheritance pattern of this disease?

What is the most common inherited hypercoagulable state? Inheritance pattern of this disease?

Answer 7

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Question 8

MOA of desmopressin for treatment of von-wilebrand disease?

Answer 8

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Question 9

Two possible cases that can cause plasmin overactivity? treatment?

Answer 9

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treated with aminocaproic acid, same one used to reverse plasmin overactivity

• Plasmin overactivity -> excessive cleavage of fibrin, coagulation factor degradation, inhibition of platelet aggregation

Question 10

What does lines of Zahn indicate?

Answer 10

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Question 11

classic physical exam in fat embolism?

Answer 11

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Question 12

Why PE is most often clinically silent?

Answer 12

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Question 13

“Gastrectomy”. what two hematologic disorders can this buzzword suggest?

Answer 13

1. iron deficiency anemia: gastric acid is required to make iron as Fe2+ form, absorbable state of iron
   ( also vitamin C)
2. megaloblastic anemia due to B12 deficiency

Question 14

List 4 stages of iron deficiency anemia in chronological order

Answer 14

1.
2.
3.
4.

Question 15

3 phenotypes of Plummer-Vinson syndrome?

Answer 15

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Question 16

2 MOA of hepcidin that results in anemia

Answer 16

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Question 17

Anemia of chronic disease

• % saturation
• TIBC
• serum iron
• Free protoporphyrin

Answer 17

• % saturation
• TIBC
• serum iron
• Free protoporphyrin

Question 18

Where does iron is stained with perussian-blue staining exactly?

• nucelus?
• cytoplsam?
• mitochondria?

Answer 18

mitochondria

• Iron gets trapped WITHIN mitochondria. As mitochondria is normally present peripherally around nucleus, it presents as ring.
• Iron laden mitochondria

Question 19

Three acquired causes of sideroblastic anemia?

Answer 19

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Question 20

How many alpha globulin genes? what chromosome?

Answer 20

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Question 21

How many beta globulin genes? what chromosome?

Answer 21

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Question 22

What is Hb Barts?

Answer 22

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Question 23

Nature of gene defect in alpha thalassemia? what about beta thalessemia?

Answer 23

• alpha thalassemia

- beta thalessemia?

Question 24

Measuring level of what metabolite will be helpful for distinguishing megaloblastic anemia due to B12 deficiency vs. B9 deficiency?

Answer 24

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Question 25

What is the color of reticulocyte in blood smear? why?

Answer 25

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Question 26

How does anemia alter reticulocyte count? What is the formula to correct reticulocyte count?

Answer 26

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Question 27

MCHC (mean corpuscular hemoglobin concentration) in hereditary spherocytosis?

Answer 27

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Question 28

Most common cause of death in sickle cell in - kids - adults

Answer 28

- kids | - adults

Question 29

Normally sickle cell trait is not really symptomatic (as > 50% of HbS is required for sickling), EXCEPT this phenotype. What is this?

Answer 29

- * This is UWORLD question

Question 30

What is metabisulfite screen?

Answer 30

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Question 31

Inheritance pattern of PNH (Paroxysmal Nocturnal Hemogluboinuria)?

Answer 31

acquired defect (not inherited) in myeloid stem cell

Question 32

Which CD marker on neutrophil represents left shift? what is normal role of this marker?

Answer 32

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Question 33

What hematologic neoplasm is associated with eosinophilia? What is mechanism?

Answer 33

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Question 34

What hematologic neoplasm is associated with basophilia?

Answer 34

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Question 35

Infectious mononucelosis - classic symptoms (4) - EBV infects what cells through what CD marker? - Which type of atypical lymphocytes will be produced? - Where does production of atypical lymphocytes happen (2)? how does this explain symptoms?

Answer 35

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Question 36

What is major complication of infectious mononucelosis? Because of this complication, what advice should be given to patient?

Answer 36

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Question 37

What hematologic malignancy can happen as a complication of infectious mononucleoisis?

Answer 37

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Question 38

What is marker for ALL? What is function of this marker?

Answer 38

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Question 39

What is treatment for ALL? Prophylaxis to which two structures is required? why?

Answer 39

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Question 40

Why is APL is considered as medical emergency?

Answer 40

risk for DIC myleoblast contains bunch of granules, that increase coagulation *M in STOP Making New Thrombi M-malignancy

Question 41

What are two subtypes of AML that lack MPO? What is special feature of each?

Answer 41

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Question 42

What is complication of CLL?

Answer 42

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Question 43

Marker of hairy cell leukemia?

Answer 43

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Question 44

Hairy cell leukemia results in splenomegaly by accumulating in what part of spleen?

Answer 44

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Question 45

multiple myeloma and adult T cell leukemia both can cause lytic bone lesions/ hypercalcemia. What clinical features (2) suggests that it is adult T cell leukemia, but not multiple myeloma?

Answer 45

- IV drug use history (HTLV-1) | - skin rash (subcutenous infiltration of leukemic T cells)

Question 46

What is Pautrier microabscesses in mycosis fungoides?

Answer 46

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Question 47

To what other malignancy can CML develop into?

Answer 47

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Question 48

Increased of what cell is responsible for itching after bath in polycythemia vera?

Answer 48

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Question 49

Is lymphadenopathy painful or painless in - Hodgkin lymphoma - Non-Hodgkin lymphoma

Answer 49

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Question 50

Lymphadenopathy: what diseases are associated with hyperplasia of - follicle - paracortex - medullarly sinus

Answer 50

- follicle: - paracortex: - medulla:

Question 51

What is in star (white spot) of starry-sky histology of Burkitt lymphoma?

Answer 51

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Question 52

Hodgkin lymphoma - which subtype has the best prognosis? - which subtype has the worst prognosis? - which subtype is common in female? - which subtype is associated with eosinophilia? what is mechanism for eosinophilia?

Answer 52

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