Hematology

Question 1

What is the
most likely diagnosis for the patient with hemolytic anemia and hepatic thrombosis?

Answer 1

G6PD deficiency

Question 2

What condition was suspected when a patient’s hematocrit dropped from 34% to 22%?

Answer 2

Aplastic crisis due to parvovirus B19

Question 3

What are the key management strategies for sickle cell disease during a crisis?

Answer 3

• Oxygen
• hybdroxyurea
• blood transfusion
• antibiotics.

Question 4

What are the
major complications of sickle cell disease?

Answer 4

Acute chest syndrome

stroke

Moyamoya disease.

Question 5

What preventive measures are recommended for patients with sickle cell disease?

Answer 5

Vaccination and genetic counseling to prevent homozygous births.

Question 6

What is the
management protocol for acute chest syndrome in sickle cell disease?

Answer 6

• oxygen
• hydration
• analgesia
• antibiotics such as ceftriaxone or
  levofloxacin

Question 7

What is Moyamoya disease and how is it related to sickle cell disease?

Answer 7

Moyamoya disease involves
abnormal capillaries in the brain, leading to a risk of hemorrhagic stroke in sickle cell patients.

Question 8

What role does genetic testing play in managing sickle cell disease?

Answer 8

Genetic testing helps prevent homozygous births and is important for family screening and prenatal diagnosis.

Question 9

How does hydroxyurea help manage sickle cell disease?

Answer 9

Hydroxyurea increases fetal hemoglobin production, reducing pain crises and severe anemia.

Question 10

What is a common cause of transfusion-related hypotension in IGA-deficient patients?

Answer 10

Anaphylaxis due to recipient IGA antibodies

Question 11

How soon can symptoms of
transfusion-related acute lung injury
(TRALI) appear after transfusion?

Answer 11

Within six hours

Question 12

What is a significant non-cardiogenic pulmonary complication caused by donor anti-leukocyte antibodies during transfusion?

Answer 12

TRALI (Transfusion-related acute lung injury)

Question 13

What is the role of
ACE inhibitors in transfusion-related reactions?

Answer 13

They can cause
transient hypertension due to increased bradykinin levels.

Question 14

Which transfusion reaction presents with fever, chills, and septic shock within minutes of transfusion?

Answer 14

Bacterial sepsis.

Question 15

What is the primary cause of TRALI?

Answer 15

Donor antibodies against recipient leukocytes

Question 16

What are the key symptoms of thrombotic
thrombocytopenic purpura (TTP)?

Answer 16

Fever, confusion, low platelets, and elevated urea and creatinine

Question 17

What is a
distinguishing feature between TTP and immune thrombocytopenic purpura (ITP)?

Answer 17

Neurological
symptoms and fever in TTP

Question 18

Which genetic mutations are commonly tested in hereditary thrombophilia?
.

Answer 18

Factor V Leiden and prothrombin gene mutation

Question 19

What treatment is used for managing thrombotic
thrombocytopenic purpura (TTP)?

Answer 19

Plasmapheresis

unfractionated heparin

rituximab.

Question 20

What neurological symptoms can B12
deficiency cause?

Answer 20

reversible neurological symptoms like confusion, delirium, dementia, and optic atrophy
irreversible spinal cord damage affecting pyramidal tracts and posterior columns.

Question 21

How is severe megaloblastic anemia related to B12 treated?

Answer 21

intramuscular B12 injections. Monitoring for hypokalemia and rebound thrombocytosis is important during treatment.

Question 22

What are the common causes of folate deficiency?

Answer 22

Folate deficiency can result from

• poor diet
• alcohol abuse
• malabsorption
• certain medications (e.g., methotrexate)

Question 23

What are the symptoms of folate deficiency?

Answer 23

• macrocytic anemia
• jaundice
• glossitis
• angular stomatitis

Question 24

Why is it important to rule out B12
deficiency when diagnosing folate deficiency?

Answer 24

Treating folate deficiency without addressing a concurrent B12
deficiency can worsen neurological symptoms

Question 25

What is the treatment for folate deficiency?

Answer 25

Treatment includes oral folic acid supplements with a maintenance dose once deficiency is corrected.

Question 26

What are the steps involved in thrombus formation?

Answer 26

endothelial injury, vasoconstriction, platelet activation, and aggregation, followed by conversion of fibrinogen to fibrin to stabilize the clot.

Question 27

What is the difference between primary and secondary hemostasis?

Answer 27

Primary hemostasis refers to platelet aggregation, while secondary hemostasis involves the conversion of fibrinogen to fibrin.

Question 28

What role does von Willebrand factor (vWF) play in clotting?

Answer 28

VWF helps platelets adhere to the collagen in the damaged endothelium, promotes aggregation.

Question 29

Describe the intrinsic pathway of the coagulation cascade

Answer 29

The intrinsic pathway begins with 𝒇𝒂𝒄𝒕𝒐𝒓 VII activation, leading to the activation of 𝒇𝒂𝒄𝒕𝒐𝒓 𝑿𝑰 and 𝑰𝑿, and ultimately 𝑿 with 𝒇𝒂𝒄𝒕𝒐𝒓 𝑽𝑰𝑰𝑰 and 𝒄𝒂𝒍𝒄𝒊𝒖𝒎 playing critical roles.

Question 30

What is the extrinsic pathway and which factors are involved?

Answer 30

The extrinsic pathway involves 𝒕𝒊𝒔𝒔𝒖𝒆 𝒇𝒂𝒄𝒕𝒐𝒓 ( 𝑻𝑭) and 𝒇𝒂𝒄𝒕𝒐𝒓 𝑽𝑰𝑰, which activate 𝒇𝒂𝒄𝒕𝒐𝒓 𝑿. It primarily affects 𝒇𝒂𝒄𝒕𝒐𝒓 II, VII, 𝑰𝑿 and 𝑿 produced by the liver

Question 31

What are the differences between Hemophilia A, B, and C?

Answer 31

Hemophilia A is caused by factor VIII deficiency, Hemophilia B by factor 𝑰𝑿 deficiency, and Hemophilia C by factor 𝑿𝑰 deficiency, all of which prolong PTT.

Question 32

What are the differences between Hemophilia A, B, and C?

Answer 32

Hemophilia A is caused by factor 𝑽𝑰𝑰𝑰 deficiency, Hemophilia B by factor 𝑰𝑿 deficiency, and Hemophilia C by factor 𝑿𝑰 deficiency, all of which prolong PTT

Question 33

How is Hemophilia A diagnosed?

Answer 33

Hemophilia A is diagnosed through a prolonged PTT and low factor VIII activity, with normal PT and platelet count

Question 34

When is heparin preferred over DOACS?

Answer 34

Heparin is used in settings like pregnancy, severe renal failure, or immediate anticoagulation needs, while DOACs are typically used for long-term management.

Question 35

What is the role of protamine sulfate in heparin use?

Answer 35

Protamine sulfate is an antidote to heparin, reversing its anticoagulant effects in cases of overdose or excessive bleeding.

Question 36

What role does vitamin K play in coagulation?

Answer 36

Vitamin K is essential for synthesizing clotting factors II, VII, IX, and X, which are part of both the extrinsic and common pathways

Question 37

What is the treatment for warfarin overdose with a high INR?

Answer 37

Treatment includes stopping warfarin, administering vitamin K (IV for severe cases), and possibly using prothrombin complex concentrate (PCC) to rapidly reverse coagolopathy

Question 38

What is the role of tranexamic acid in bleeding management?

Answer 38

Tranexamic acid inhibits plasminogen activation, stabilizing clots and is used in cases like postpartum hemorrhage and trauma-related bleeding

Question 39

Why does B12 deficiency lead to subacute combined degeneration of the spinal cord?

Answer 39

B12 is crucial for myelin synthesis. Its deficiency impairs the methylation process, leading to demyelination of the dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts

Question 40

What is Lhermitte's sign and its association with B12 deficiency?

Answer 40

Lhermitte's sign is an electric shock-like sensation running down the spine with neck flexion. It is associated with B12 deficiency due to dorsal column involvement