Patho-RBC and Bleeding Disorders Flashcards

1
Q

Erythropoeitin level in hemolytic anemias

A

Increased

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2
Q

Most common cause of IDA overall?

A

GI bleeding

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3
Q

Intrinsic hemolysis is caused by

A

Intrinsic defects in rbc

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4
Q

How do u differentiate extravascular from intravascular hemolysis?

A

Extravascular hemolysis has splenomegaly

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5
Q

What is the expected morphology on peripheral blood smear of hemolytic anemias?

A

Normicytic normochromic

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6
Q

Hereditary spherocytosis is brought about by what mutations

A

Ankyrin
Spectrin
Band 3
Band 4.2

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7
Q

In a patient with hereditary spherocytosis, infection with parvovirus b19 can cause?

Infection with EBV?

A

Parvo - aplastic crisis

EBV- hemolytic crisis

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8
Q

Small dark nuclear remnants present in RBCs of asplenic patients

A

Howell-jolly bodies

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9
Q

G6pd is the rate limiting step in which biochemical pathway?

A

Pentose phosphate pathway

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10
Q

What is the function of G6PD

A

Reduces NADP to NADPH; provides reducing equivalents

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11
Q

Morphology G6PD deficiency

A

Heinz bodies

Bite cells

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12
Q

What are heinz bodies?

A

Membrane bound precipitates on denatured globin chains

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13
Q

Mutation in sickle cell anemia

A

Point mutation in 6th codon of beta globin

Leads to

Replacement of glutamate with VALINE

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14
Q

Dehydrated RBCs with bulls eye apoearance seen in sickle cell disease

A

Codocytes

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15
Q

Why are infants with sickle cell disease asymptomatic until 2-4 mos of age?

A

Due to high fetal hemoglobin F

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16
Q

Crises in Sickle cell anemia

A

Vasoocclusive crisis
Sequestration crisis
Aplastic crisis

17
Q

Most common manifestation of vasoocclusive crisis in sickle cell anemia?

A

Hand foot syndrome

Inflammation of finger and toe

18
Q

Massive entrapment of sickle cells leads to rapid splenic enlargement, hypovolemia, and shock

A

Sequestration crisis

19
Q

Renal manifestation of sickle cell anemia

A

Hyposthenuria

Inability to concentrate urine

20
Q

Signs of extramedullary hematopoieisis in sickle cell

A

Crew cut skull

Prominent cheekbones

21
Q

What is the best initial diagnostic test for sickle cell anemia?

A

PBS

Not used in newborns

22
Q

What is the drug of choice for sickle cell anemia and what is its mechanism of action?

A

Hydroxyurea (increases HbF)

23
Q

Diagnostic gold standard for sickle cell anemia

A

Hemoglobin electrophoresis