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Normocytic anemia Flashcards

1
Q

When a Pt presents with early concurrent B12 or folate deficiency and iron deficiency, what type of anemia is found?

A

Normocytic anemia

Microcytic anemia will present as time goes on

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2
Q

Rapid decline in the Hb/hematocrit w/o hyperbilirubinemia

A

Blood loss

Anemia may not show until expansion of blood volume w/ plasma/IV fluid

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3
Q

Blood loss into an extravascular space can masquerade as ______

A

Hemolysis, because breakdown of the extravascular blood collection will lead to an increase in indirect bilirubin.

This would be a very characteristic scenario for an elderly person who presents with a broken hip, a drop in hematocrit/hemoglobin, and elevated bilirubin.

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4
Q

Lab findings in hemolysis

A

Hyperbilirubinemia (indirect)

Elevated lactacte DH (LDH)

Decreased haptoglobin

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5
Q

Anemia associated w/ congenital RBC enzyme deficiency

A

Intravascular hemolytic anemia

G6PD deficiency
Pyruvate kinase deficiency
Hexokinase deficiency

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6
Q

Non-immune intravascular hemolysis caused by infections

A
  1. Clostridium septicum
  2. Malaria
  3. Leishmaniasis
  4. Babesiosis
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7
Q

Types of non-immune hemolysis

A

Mechanical hemolysis

Microangiopathic hemolysis

Specific infections

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8
Q

Causes of extravascular hemolysis

A

RBC membrane disorders: hereditary spherocytosis; hereditary ovalocytosis

Autoimmune hemolytic anemia - most types (“warm” AIHA)

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9
Q

Hemoglobinuria is indicative of what type of anemia?

W/o hematuria

A

Intravascular hemolytic anemia

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10
Q

Plasma hemoglobinemia is specific to?

A

Intravascular hemolysis

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11
Q

Urine hemosiderin
is a specific marker of?

A

Chronic intravascular hemolysis

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12
Q

Sickle Hb polymerization leads to what pathophysiological trait of sickle cell anemia?

A

Vasoocclusion

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13
Q

Perfusion/repurfusion leads to what pathophysiological trait of sickle cell anemia?

A

Inflammatory cytokine activation

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14
Q

Intravascular hemolysis leads to what pathophysiological trait of sickle cell anemia?

A

Free Hb that binds to NO - creating vasodilation and pulmonary hypertension

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15
Q

Increased expression of RBC adhesion molecules in sickle cell anemia leads to?

A

Vasoocclusion

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16
Q

Virtually all Pt’s w/ SS disease undergo?

A

Auto-infarction and atrophy of the spleen early in childhood - increases susceptibility to infection by encapsulated organisms

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17
Q

Transient aplastic crisis

A

An acute severe (but self-limited) drop in hemoglobin with reticulocytopenia due to acute parvovirus B19 infection of Pt w/ sickle cell anemia

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18
Q

Splenic sequestration (“hyperhemolytic crisis”) crisis

A

Associated with delayed hemolytic transfusion reactions.

  1. Acute splenomegaly
  2. massive drop in hemoglobin concentration
  3. extremely high reticulocyte counts

Seen in sickle cell anemia

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19
Q

Cholestatic crisis

A

1.Severe abdominal pain
2.Bilirubin > 50 mg/dL (normal ~1.3 mg.dL)
3.Hepatocellular damage

Seen in sickle cell anemia

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20
Q

The optimal hemoglobin concentration in an SS patient

A

9-10 mg/dL

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21
Q

Nearly all Pt’s w/ hereditary membrane defects will eventually require _____

A

Cholecystectomy

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22
Q

Inheritance pattern of heriditary spherocytosis (HS)

A

Autosomal dominant

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23
Q

Most common RBC membrane defect resulting in hemolytic anemia

A

Hereditary spherocytosis

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24
Q

Eosin-5-maletimide binds to what on RBCs?

Provides definitive confirmation for what Dx?

A

EMA binds to AEP/band3 on RBCs

Dx: Hereditary spherocytosis

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25
Q
A

Hereditary stomatocytosis

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26
Q

What is “absolutely contraindicated” in hereditary stomatocytosis?

A

Splenectomy - extremely high incidence of post-splenectomy intra-abdominal venous thrombosis

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27
Q

LOF mutation in PIGA leads to?

A

Paroxysmal nocturnal hemoglobinuria (PNH)

PIGA gene regulates the anchoring proteins to the RBC cell membrane by glycosylphosphatidylinositol - one of the proteins is decay accelerating factor (DAF)

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28
Q

An acquired clonal disorder where patients have hemolytic anemia, frequently with leukopenia and often thrombocytopenia and a hypocellular bone marrow.

A

Paroxysmal nocturnal hemoglobinuria (PNH)

Also increased risk of venous thromboembolic events

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29
Q

Decay accelerating factor (DAF)

A

Turns off complement on the cell surface

PIGA LOF mutation in PNH - RBCs are excessively susceptible to complement-mediated lysis

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30
Q

How is paroxysmal nocturnal hemoglobinuria dx’d?

A

Demonstrating decreased expression of the glycosylphosphatidylinositol-anchored proteins CD55 and CD59 on RBC and granulocyte surfaces

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31
Q

Tx of paroxysmal nocturnal hemoglobinuria

A

PNH Pt’s are Tx’d w/ anti-C5a agents - eculizumab - significantly reduces hemolysis and improves QOL

require vaccination against significant organisms whose clearance is dependent on complement, especially Neisseria meningitidis and Haemophilus influenzae

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32
Q

Spur cell anemia

A

Acquired membrane defect due to dyslipidemia in end stage liver disease

As opposed to the usual target cells of liver disease, these patients develop acanthocytes

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33
Q

Spur cell anemia with 5% acanthocytes

A

predictor of short survival and is a reason for liver transplantation

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34
Q

McLeod syndrome

A

Congenital abetalipoprotinemia

X-linked abnormality of the Kell minor blood group associated w/ hemolysis, cardiomyopathy, and both peripheral and central neurologic abnormalities

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35
Q

G6PD A variants

A

commonly seen in Black individuals

These variants have significantly low levels of G6PD, primarily in older RBCs
-after the initial drop in hemoglobin/hematocrit, they will begin to recover from anemia even if the offending agent continues
-they will replete the hemolyzed cells with new younger cells

(although they will not fully recover from the anemia until the oxidative insult is removed).

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36
Q

G6PD B variants

A

characteristic variant of Mediterranean ancestry

significantly low levels in all
but reticulocytes and the very youngest RBCs, so they have more severe and sustained anemia.

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37
Q

CNSHA variants

A

Chronic nonspherocytic
hemolytic anemia

Very low baseline G6PD activity

typically present with neonatal jaundice - mildly anemic (~9-10 g/dL) all the time

develop gallstones like other patients with chronic congenital hemolysis.

If exposed to an oxidative drug, their anemia becomes much worse.

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38
Q

Inheritance pattern of G6PD deficiency

A

X-linked recessive

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39
Q

Pyruvate kinase deficiency inheritance pattern

A

Autosomal recessive

40
Q

PK deficiency sxs

A

Congenital chronic hemolytic anemia

Often beginning w/ neonatal jaundice

Develop gallstones and splenomegaly

May have extremely brisk reticulocytosis (mitigates PK deficiency - able to generate ATP from oxidative phosphorylation)

PK deficiency exhibits both intravascular and extravascular hemolysis

41
Q

Mitapivat

A

Activator of pyruvate kinase - can improve hemoglobin concentration in pyruvate kinase deficiency

42
Q

Differences between PK and hexokinase deficiencies

A

RBC 2,3- BPG levels are decreased in hexokinase deficiency.

This means that hemoglobin holds on to oxygen rather than releasing it to peripheral tissue and anemia symptomatology is more severe than would be
predicted from the hemoglobin/hematocrit.

43
Q

Causes of mechanical hemolysis

A

aka “macroangiopathic anemia”

44
Q

Disseminated intravascular coagulation (DIC)

A

coagulation is inappropriately activated by some concurrent illness – sepsis typically, but sometimes-metastatic cancer.

Platelets and soluble coagulation factors are consumed - results in prolonged clotting times and thrombocytopenia

Hemolytic anemia is not a major feature

Microangiopathic hemolytic anemia

45
Q

Cause of thrombotic thrombocytopenic purpura (TTP)

A

ADAMSTS-13 deficiency - acquired autoimmune deficiency mediated by antibodies.

Platelets are excessively activated and form disseminated small clots in the microvasculature

ADAMSTS-13 is a matrix metalloproteinase that cleaves very high molecular weight multimers of vWF

46
Q

Sxs of thrombotic thrombocytopenic purpura (TTP)

A

Hemolysis (small microvascular platelet thrombi damage passing RBCs)

Thrombocytopenia

Neurologic and renal issues

Clotting times are normal

47
Q

Hemolytic uremic syndrome (HUS)

A

Results from the activities of bacterial toxins:
-particularly Shiga toxin and the toxins from E. coli 0157 or other enterotoxigenic E. coli

48
Q

Atypical HUS (aHUS)

A

Resembles hemolytic uremic syndrome (HUS) but is caused by excessive complement activation - Tx’d w/ anticomplement agents (same as PNH)

49
Q

aHus/Hus in pregnancy

A

pregnancy-related HELLP syndrome

similar complement mediated syndrome as seen in aHUS

H - hemolysis
E - elevated
L - liver tests
L - low
P - platelets

50
Q

What infections can cause intravascular non-immune hemolysis?

A

Plasmodium falciparum (“blackwater fever) malaria

Clostridium perfringens

African trypanosomiasis (not American - Chaga’s disease)

Babesiosis

Bartonelosis

Visceral leishmaniasis

Clostridial sepsis can cause extremely rapid onset of massive hemolysis.

51
Q

Infection associated with hemophagocytosis by circulating macrophages/monocytes

A

Leishmaniasis

52
Q

First line treatment for IgG-mediated AIHA

A

Corticosteroids (prednisone) with or without rituximab (anti-CD20 mAbs)

Splenectomy = third line Tx

53
Q

First line treatment for C3-mediated AIHA

A

Rituximab (anti-CD20 mAbs)

Pt’s do not respond well to prednisone or splenectomy

54
Q

Direct Ab test (DAT - aka direct Coomb’s test) for neoantigen/immune complex

A

Only positive for C3

55
Q

Positive DAT (direct Coomb’s test) without anemia

A

Nonspecific and clinically irrelevant binding of antibodies to RBCs resulting in a positive DAT

Nonspecific finding

56
Q

Screening for alloimmunization for blood transfusion

A

Indirect antiglobulin test (aka indirect Coomb’s test)

57
Q

ABO blood group incompatibility

A

Massive hemolytic rx’n

Hemoglobinuria
Flank pain
Kidney damage

Systemic collapse and possibly death

Pt is managed w/ hydration, dialysis, and aggressive support

58
Q

Delayed hemolytic transfusion reaction (DHTR)

A

At time of transfusion - IAT was negatigve

Approx. 5-7 days later - sudden drop in Hb/Hct with signs of hemolysis (IAT is not positive)

Pt was exposed to Ag on transfused blood from a prior pregnancy/transfusion

The DAT is negative because
the only RBCs to which the antibody would bind would be the transfused RBCs and those had been hemolyzed.

The “hyperhemolytic crisis” seen in sickle-cell patients is an unusually severe variant of DHTR.

59
Q

Pure red cell aplasia (PRCA)

A

Marrow failure state

Presents as an isolated normocytic anemia w/ extreme reticulocytopneia (uncorrected reticulocyte count frequently < 0.1%; absolute reticulocyte count typically <10,000/µL).

PRCA is usually a primary autoimmune disorder mediated by an IgG antibody against some erythroid progenitor antigen that has not yet been defined.

It can also be secondary to other disorders, most significantly chronic lymphocytic leukemia; systemic lupus erythematosus; thymoma; or B19 parvovirus infection in an immunocompromised patient, particular organ transplant patients or patients with HIV infection.

60
Q

Anemia of chronic kidney disease

A

Anemia is almost invariable in significant renal insufficiency, beginning when the estimated glomerular filtration rate (eGFR) falls below 45 mL/min/1.73 m2 body surface area.

Anemia is a less frequent
complication of polycystic kidney disease, due to secretion of erythropoietin (EPO) from the cysts.

Echinocytes (“burr cells”) may be seen, particularly with elevated blood urea nitrogen (BUN). They look like acanthocytes but the cytoplasmic projections are shorter and blunted
61
Q

Tx of anemia of uremia

A

Anemia of uremia aka anemia of CKD

Tx w/ recombinant EPO or an EPO analog

Fe supplementation when Tf/TIBC saturation is less than 30% or serrum ferritin concentration is less than 500 ng/mL

62
Q

Which of the following drugs inhibits sickle RBC adhesion to the vascular endothelium?

A. Hydroxyurea
B. Crizanlizumab
C. L-glutamine

A

B. Crizanlizumab

L-glutamine reduces inflammation
Hydroxyurea increases HbF conc.

63
Q

In which of the following syndromes is the etiology of hemolysis extrinsic to the RBC?

A. Spur cell anemia
B. Delayed hemolytic transfusion rx’n
C. Methylene blue exposure in a Pt w/ G6PD deficiency
D. Paroxysmal nocturnal hemoglobinuria

A

B. Delayed hemolytic transfusion reaction

64
Q

Hereditary spherocytosis most commonly results from mutations in the genes for which RBC proteins?

A

Both anykrin and spectrin

Band 7 defect gives you hereditary stomatocytosis

65
Q

Which of the following would be most likely to be associated with sickle cell trait (AS genotype)?

A. Painful vasoocclusive
B. Auto-infarction of the spleen in childhood
C. Dilute urine specific gravity gravity despite fluid fluid retention
D. Splenomegaly

A

C. Dilute urine specific gravity despite fluid restriction

Sickle trait Pt’s do not get painful vasoocclusive crises

This, episodic hematuria (due to papillary necrosis), and increased risk of a rare kidney cancer are the only sxs of Pt w/ sickle trait

66
Q

A 48 year old man has received repeated RBC transfusions for myelodysplastic syndrome. Five days after his most recent transfusion he has a sudden drop in hemoglobin and hematocrit with increased in serum bilirubin. There is no evidence of bleeding.

Immunohematologic evaluation of this patient is most likely to have which of the following results for direct/indirect antiglobulin tests (Coomb’s test)?

A

Negative direct antiglobulin test

Positive indirect antiglobulin test

67
Q

Hemolysis in which of the following disorders is predominantly intravascular?

A. Clostridium sepsis
B. Hereditary spherocytosis
C. IgG “warm” autoimmune hemolytic anemia
D. Spur cell anemia

A

A. Clostridium sepsis

68
Q

Which of the following is most consistent with Gilbert syndrome?

A. Elevated direct bilirubin
B. Elevated indirect bilirubin
C. Decreased haptoglobin

A

B. Elevated indirect bilirubin

69
Q
A

D. GI blood loss

70
Q

A. Folate supplementation
B. Aortic valve repair
C. Splenectomy
D. Supplemental oxygen

A

A. Folate supplementation

71
Q

Direct vs. indirect antiglobulin test (Coomb’s)

A
72
Q

What type of sickle cell anemia is implicated when a Pt presents w/ chronic splenomegaly

Not an acute crisis

A

Sickle C or sickle-thalassemia

73
Q
A

A. Serum iron, transferrin/TIBC, ferritin

74
Q
A

C. Decreased serum haptoglobin

Signs of hemolysis
75
Q
A

D. Increased urine hemoglobin

Urine hemosiderin = chronic intravascular hemolysis (heme deposited in renal tubule

76
Q
A

C. PK deficiency

77
Q
A

C. L-glutamine

Hydroxyurea - increased HbF

Crizanlizumab - prevents cell adhesion to endothelium

78
Q

SS vs. AS in sickle cell anemia

A
79
Q

Splenic seqestration in Pt w/ sickle cell anemia

A

Typically due to delayed transufsion reaction

80
Q
A

C. Hereditary stomatocytosis

Hereditary stomatocytosis is resulting from defect in band 7 (acquired)

Pt’s w/ hereditary stomatocytosis that receive splenectomy get abdominal thromboses

81
Q
A

Hereditary spherocytosis

82
Q

Eculizumab

A

Anticomplement mAb used in Tx of paroxysmal nocturnal hemoglobinuria

Have to be vaccinated against pertient encapsulated organisms

83
Q
A

A. Increased RBC 2,3-BPG levels

84
Q
A

Blister cells and bite cells (denatured hemoglobin that does not take up stain)

Blister would stain w/ Heinz body stains

Unstable hemoglobin - resulting from defect in metabolism in RBC

85
Q
A

C. Antibodies against ADAMSTS13

E. coli O157 toxin - HUS
D - AHUS

86
Q
A

Schistocytes

87
Q

Tx for thrombotic thrombocytopenic purpura

A

Plasma exchange = #1

Caplacizumab (anti-vWF)

88
Q
A

A. Rhesus (Rh) Ag complex

I/i Ag complex - cold autoimmune hemolytic anemia

Duffy complex - receptor that takes up malaria

89
Q

Genotypes of ABO blood types

A
90
Q
A

B. Anemia of CKD

91
Q

Masked megaloblastic anemia

A

Concurrent B12 and Fe deficient - presents initially as normocytic the progresses to microcytic

92
Q

Why are Pt’s w/ polycystic kidney disease rarely EPO deficient?

A

Renal cysts contain EPO that can diffuse into systemic circulation

93
Q

Physiologic anemia of pregancy

A

During pregnancy - RBC mass goes up, but slower than plasma volume

94
Q
Marrow smear
A

Pure RBC aplastic anemia

95
Q

Infection of pronormoblast should make you think

A

Parvovirus

Tx’d w/ IVIG

96
Q

Thymoma can be associated w/?

Two pathologies

A

Pure red cell aplasia

Myasthenia gravis

MI Immuno/Heme (35 decks)

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