Hematology Flashcards
What is the
most likely diagnosis for the patient with hemolytic anemia and hepatic thrombosis?
G6PD deficiency
What condition was suspected when a patient’s hematocrit dropped from 34% to 22%?
Aplastic crisis due to parvovirus B19
What are the key management strategies for sickle cell disease during a crisis?
- Oxygen
- hybdroxyurea
- blood transfusion
- antibiotics.
What are the
major complications of sickle cell disease?
Acute chest syndrome
stroke
Moyamoya disease.
What preventive measures are recommended for patients with sickle cell disease?
Vaccination and genetic counseling to prevent homozygous births.
What is the
management protocol for acute chest syndrome in sickle cell disease?
- oxygen
- hydration
- analgesia
- antibiotics such as ceftriaxone or
levofloxacin
What is Moyamoya disease and how is it related to sickle cell disease?
Moyamoya disease involves
abnormal capillaries in the brain, leading to a risk of hemorrhagic stroke in sickle cell patients.
What role does genetic testing play in managing sickle cell disease?
Genetic testing helps prevent homozygous births and is important for family screening and prenatal diagnosis.
How does hydroxyurea help manage sickle cell disease?
Hydroxyurea increases fetal hemoglobin production, reducing pain crises and severe anemia.
What is a common cause of transfusion-related hypotension in IGA-deficient patients?
Anaphylaxis due to recipient IGA antibodies
How soon can symptoms of
transfusion-related acute lung injury
(TRALI) appear after transfusion?
Within six hours
What is a significant non-cardiogenic pulmonary complication caused by donor anti-leukocyte antibodies during transfusion?
TRALI (Transfusion-related acute lung injury)
What is the role of
ACE inhibitors in transfusion-related reactions?
They can cause
transient hypertension due to increased bradykinin levels.
Which transfusion reaction presents with fever, chills, and septic shock within minutes of transfusion?
Bacterial sepsis.
What is the primary cause of TRALI?
Donor antibodies against recipient leukocytes
What are the key symptoms of thrombotic
thrombocytopenic purpura (TTP)?
Fever, confusion, low platelets, and elevated urea and creatinine
What is a
distinguishing feature between TTP and immune thrombocytopenic purpura (ITP)?
Neurological
symptoms and fever in TTP
Which genetic mutations are commonly tested in hereditary thrombophilia?
.
Factor V Leiden and prothrombin gene mutation
What treatment is used for managing thrombotic
thrombocytopenic purpura (TTP)?
Plasmapheresis
unfractionated heparin
rituximab.
What neurological symptoms can B12
deficiency cause?
reversible neurological symptoms like confusion, delirium, dementia, and optic atrophy
irreversible spinal cord damage affecting pyramidal tracts and posterior columns.
How is severe megaloblastic anemia related to B12 treated?
intramuscular B12 injections. Monitoring for hypokalemia and rebound thrombocytosis is important during treatment.
What are the common causes of folate deficiency?
Folate deficiency can result from
- poor diet
- alcohol abuse
- malabsorption
- certain medications (e.g., methotrexate)
What are the symptoms of folate deficiency?
- macrocytic anemia
- jaundice
- glossitis
- angular stomatitis
Why is it important to rule out B12
deficiency when diagnosing folate deficiency?
Treating folate deficiency without addressing a concurrent B12
deficiency can worsen neurological symptoms
What is the treatment for folate deficiency?
Treatment includes oral folic acid supplements with a maintenance dose once deficiency is corrected.
What are the steps involved in thrombus formation?
endothelial injury, vasoconstriction, platelet activation, and aggregation, followed by conversion of fibrinogen to fibrin to stabilize the clot.
What is the difference between primary and secondary hemostasis?
Primary hemostasis refers to platelet aggregation, while secondary hemostasis involves the conversion of fibrinogen to fibrin.
What role does von Willebrand factor (vWF) play in clotting?
VWF helps platelets adhere to the collagen in the damaged
endothelium, promotes aggregation.
Describe the
intrinsic
pathway of the coagulation cascade
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.
What is the
extrinsic pathway and which factors are involved?
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
What are the differences between
Hemophilia A, B, and C?
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.
What are the differences between
Hemophilia A, B, and C?
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
How is Hemophilia A diagnosed?
Hemophilia A is diagnosed through a prolonged PTT and low factor VIII activity, with normal PT and platelet count
When is heparin preferred over
DOACS?
Heparin is used in settings like pregnancy, severe renal failure, or immediate anticoagulation needs, while DOACs are typically used for
long-term management.
What is the role of protamine sulfate in heparin use?
Protamine sulfate is an antidote to
heparin, reversing its anticoagulant effects in cases of overdose or excessive bleeding.
What role does vitamin K play in coagulation?
Vitamin K is essential for synthesizing clotting factors II, VII, IX, and X, which are part of both the extrinsic and common
pathways
What is the treatment for warfarin overdose with a high INR?
Treatment includes stopping warfarin, administering vitamin K (IV for severe cases), and possibly using prothrombin complex
concentrate (PCC) to rapidly reverse coagolopathy
What is the role of tranexamic acid in bleeding management?
Tranexamic acid inhibits plasminogen activation, stabilizing clots and is used in cases like postpartum hemorrhage and trauma-related bleeding
Why does B12 deficiency lead to subacute combined degeneration of the spinal cord?
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
What is Lhermitte’s sign and its association with B12 deficiency?
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