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Hematology and Oncology ERIN > Normocytic anemias > Flashcards

Normocytic anemias Flashcards

1
Q

Normal reticulocytes count

A

1-2%

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2
Q

How should bone marrow properly respond to anemia?

A

Increase reticulocyte count >3%

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3
Q

How to correct reticulocyte count

A

RC x (Hematocrit/45)

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4
Q

Clinical findings and lab findings of extravascular hemolysis

A

Anemia with spenomegaly
Jaundice (due to unconjugated bilirubin)
Increased risk for bilirubin gallstones
Marrow hyperplasia and corrected reticulocyte count >3%

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5
Q

Clinical findings and lab findings of intravascular hemolysis

A

Hemoglobinemia
Hemoglobinuria
Hemosiderinuria
Decreased serum haptoglobin (free haptoglobin binds Hb)

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6
Q

What causes hereditary spherocytosis?

A

inherited defect of cytoskeleton-membrane tethering proteins most commonly ankyrin, spectrin or band 3

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7
Q

Pathophys of hereditary spherocytosis?

A

membrane blebs form and are lost over time leading to loss of membrane and spherocyte formation
Sperocytes are less able to maneuver through splenic sinusoid and are consumed by spenic macrophages results in anemia

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8
Q

clinical and lab findings of hereditary spherocytosis

A

spherocytes
Increased RDW and MCHC
Splenomegaly, jaundice with unconjugated bilirubin, increased risk for bilirubin gallstones
Increased risk for aplastic crisis

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9
Q

Diagnosis of hereditary spherocytosis

A

osmotic fragility test

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10
Q

Treatment and subsequent findings of hereditary spherocytosis

A

Splenectomy - resolves anemia

Spherocytes will persists and Howell-Jolly bodies will emerge

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11
Q

pathophys of sickle cell anemia

A

Autosomal recessive mutation in Beta chain of hemoglobin - glutamic acid –> valine
HbS polymerizes when deoxygenated; polymers aggregate into sickle cells.

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12
Q

When does sickling occur?

A

hypoxemia, dehydration, acidosis

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13
Q

treatment of sickle cell

A

hydroxyurea

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14
Q

RBC membrane damage from sickling and recycling leads to what type of hemolysis?

A

BOTH extravascular and intravascular

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15
Q

Clinical findings of sickle cell

A

Massive erythroid hyperplasia – crewcut xray of skull, chipmunk facies
Hepatomegaly from intramedullary hematopoiesis
Risk of aplastic crisis with parvovirus

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16
Q

Complications of extensive sickling in sickle cell disease

A

vaso-occlusion - dactylitis
Autosplenectomy - fibrotic spleen, increases risk of infection from encapsulated organisms
increased risk of salmonella paratyphi
Acute chest syndrome - vasoocclusion in pulmonary microcirculation
Pain crisis
Renal papillary necrosis

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17
Q

Most common cause of death in adult patients with sickle cell anemia

A

Acute chest syndrome

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18
Q

Blood findings in sickle cell trait

A

<50% HbS in RBCs, about 55% HbA, 2% HbA2

No sickle cells on blood smear

Positive metabisulfite screen

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19
Q

What is a metabisulfite screen?

A

A test used to identify cells with any amount of HbS - cells with any amount will sickle - positive in both disease and trait

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20
Q

What is hemoglobin C?

A

mutation in Beta chain of hemoglobin - glutaminic acid replaced with lysine

21
Q

Inheritance pattern of hemoglobin C?

A

autosomal recessive

22
Q

Presentation of hemoglobin C

A

mild anemia due to extravascular hemolysic

23
Q

Blood smear findings of hemoglobin C

A

HbC crystals

24
Q

Paraxysmal nocturnal hemoglobinuria pathophys

A

AQUIRED defect in myeloid stem cells causing loss of GPI, thus cells are susceptible to complement mediated damage leading to intravascular hemolysis

25
Q

Normal function of GPI

A

Ancors DAF to the surface of cells which prevents complement activation

26
Q

Why does intravascular hemolysis occur at nighttime with paroxysmal nocturnal hemoglobinuria?

A

Blood becomes mildly acidotic due to shallow breathing

27
Q

Blood findings in intravascular hemolysis

A

hemoglobinuria and hemoglobinemia

hemosiderinuria days later

28
Q

Screening test for Paraxysmal nocturnal hemoglobinuria

A

sucrose test

29
Q

Confirmatory test for Paraxysmal nocturnal hemoglobinuria

A

Acidfied serum test or flow cytometry to detect lack of DAF (CD55)

30
Q

Main cause of death in paraxysmal nocturnal hemoglobinuria

A

thrombosis of hepatic, portal or cerebral veins (destroyed platelets release contents leading to thrombosis)

31
Q

Complications of paraxysmal nocturnal hemoglobinuria

A

Iron deficiency anemia and AML

32
Q

G6PD Deficiency inheritance pattern

A

X-linked

33
Q

G6PD pathophys

A

reduced half life of G6PD results in decreased NADPH and thus decreased reduced glutathione –> oxidative injury and intravascular hemolysis

34
Q

What are the variants of G6PD deficiency?

A

African variant- mildly reduced half life of G6PD leads to mild intravascular hemolysis with oxidative stress

Mediterranean variant - markedly reduced half life of G6PD leading to marked intravascular hemolysis with oxidative stress

35
Q

What findings will you seen on histology of someone with G6PD deficiency?

A

heinz bodies(precipitated Hb) and bite cells

36
Q

Presentation of G6PD deficiency

A

back pain and hemoglobinuria hours after exposure to oxidative stress

37
Q

Diagnosis of G6PD deficiency ?

A

Heinz preparation (special stain) and enzyme study - must be performed weeks after episode (since effected cells will all be lysed during episode so only normal cells will be left)

38
Q

Immune hemolytic anemia pathophys

A

Antibodies produced against RBCs
IgG - extravascular hemolysis
IgM - intravascular hemolysis

39
Q

IgG mediated immune hemolytic anemia pathophys

A

IgG binds RBcs in warmer temps (central body) leading to consumption by splenic macrophages

40
Q

What is igG mediated immune hemolytic anemia associated with?

A

SLE
CLL
certain drugs

41
Q

treatment of igG mediated immune hemolytic anemia

A

cessation of offending agent, IVIG, steroids, if necessary a splenectomy

42
Q

igM mediated immune hemolytic anemia pathophys

A

igM binds RBCs and fixes complement in colder temps (extremities) –> RBCs inactive complement but the residual C3b causes opsonization and consumption by splenic macrophages

Excessive complement activation may lead to intravascular hemolysis

43
Q

what is igM mediated immune hemolytic anemia associated with?

A

Mycoplasma pneumonia and infectious mononucleosis

44
Q

Direct coombs test

A

Confirms presence of antibody or complement coated RBCs

45
Q

Indirect coombs test

A

confirms presence of antibodies in patients serum

46
Q

What does parvovirus B19 infect?

A

progenitor red cells — halts erythropoiesis

47
Q

What will a biopsy revel in aplastic anemia?

A

Empty, fatty bone marrow

48
Q

What is myelophthisic process?

A

pathologic process that replaces bone barrow (eg metastatic cancer)
Impairs hematopoiesis -> pancytopenia

Hematology and Oncology ERIN (12 decks)

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