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Pediatric EOR > Hematology > Flashcards

Hematology Flashcards

1
Q

Role of Haptoglobin

A
  • binds to free hemoglobin to reduce its oxidative toxicity
  • a low haptoglobin level is seen in hemolytic anemia
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2
Q

List the causes of macrocytic anemia

A
  • vitamin B 12 deficiency
  • Folate deficiency
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3
Q

What is the most common cause of vitamin B12 deficiency

A
  • pernicious anemia : malabsorption
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4
Q

A (+) schilling test is indicative of

A

pernicious anemia

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5
Q

List the most common etiologies of microcytic anemia

A
  • iron deficiency
  • alpha/beta thalassemia
  • lead poisoning
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6
Q

What would you expect serum Fe, ferritin, and TIBC levels to be in iron deficiency anemia

A
  • serum Fe: low
  • Ferritin: Low
  • TIBC: high
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7
Q

What are the normal types of hemoglobin

A
  • adult hemoglobin: make up 95% after age 6 mo.
  • HbgA (2 alpha, 2 beta)
  • HbgA2 (2 alpha, 2 delta)
  • Fetal hemoglobin: HbgF (aa/yy)
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8
Q

What is Cooley’s anemia

A
  • Beta thalassemia major
  • both Beta genes are mutated
  • patients normal at birth due to fetal hemoglobin but become symptomatic at 6 months
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9
Q

Microcyctic anemia with basophilic stippling is consistent with what condition

A

lead poisoning

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10
Q

Anemia of chronic disease usually presents with what ferritin level

A
  • increased ferritin (as an acute phase reactant)
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11
Q

G6PD deficiency is what type of genetic disorder? It primarily affects what patient population?

A
  1. x-linked recessive
  2. african americans
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12
Q

Episodic no immune acute hemolytic anemia that presents with Heinz bodies on peripheral smear

A

G6PD deficiency

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13
Q

Howell-jolly bodies are seen in what condition

A

sickle cell disease

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14
Q

Children with sickle cell should be immunized against

A
  • S. Pneumococcus
  • H. Influenzae type B
  • N. Meningococcus
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15
Q

Thrombotic Thrombocytopenic Purpura is caused by

A
  • antibodies against ADAMTS13
  • ADAMTS13 is responsible for cutting vWF -> large vWF multi eras adhere to platelets and cause endothelium platelet adhesion -> small vessel thrombosis -> RBC’s become sheared as they circulate in the partially occluded small vessels -> hemolytic anemia
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16
Q

TX of TTP

A

Plasmapharesis: removes antibodies against ADAMTS13

17
Q

What is the triad seen in hemolytic uremic syndrome

A
  • microangiopathic hemolytic anemia
  • kidney failure
  • Thrombocytopenia
18
Q

hemolytic uremic syndrome is usually preceded by infection with

A
  • E-Coli O157:H7
19
Q

What is idiopathic thrombocytes purpura

A
  • Autoimmune, antibody against platelets
  • MC seen after viral infection
20
Q

What is Hemophilia A

A
  • Factor VIII deficiency -> affects intrinsic pathway
  • x-linked
21
Q

Hemophilia A most commonly presents with what symptom

A
  • hemarthrosis: bleeding in weight bearing joints (ex: ankles)
22
Q

What is hemophilia B

A
  • factor IX deficiency
  • Christmas disease
  • X-linked
23
Q

Function of vWF

A
  • necessary for initial platelet adhesion
  • prevents Factor VIII degradation
24
Q

Leukemia is the malignancy of what type of cells?

A
  • malignancy of WBC -> bone marrow proliferation of abnormally large amounts of WBCs
  • Get pancytopenia: neutropenia, anemia, and thrombocytopenia
25
Q

What is the most common childhood malignancy

A
  • ALL: acute lymphocytic leukemia
26
Q

What is the most common form of leukemia in adults

A
  • CLL: chronic lymphocytic leukemia
27
Q

CLL is a malignancy of what type of cells

A
  • B cell clonal malignancy
28
Q

Auer rods are seen in what condition

A
  • AML: acute myeloid leukemia
29
Q

Philadelphia chromosome is seen in what condition

A
  • CML: chronic myeloid leukemia
  • Philadelphia chromosome is a translocation between chromosome 9 and 22
30
Q

Most common solid tumor in children

A
  • neuroblastoma - arise form primitive sympathetic ganglion cells
  • adrenal gland most common

Pediatric EOR (11 decks)

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