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Hematology > Chapter 129 Hemolytic Anemia And Anemia due to Acute Blood Loss > Flashcards

Chapter 129 Hemolytic Anemia And Anemia due to Acute Blood Loss Flashcards

1
Q

Main sign in patients with hemolytic anemia?

A

Jaundice

Other signs:
Pallor

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2
Q

Main symptom of patients with hemolytic anemia ?

A

Splenomegaly
(Spleen is the preferential site of hemolysis)

Other symptoms:
Frontal bossing of skull (in severe congenital cases)
Hematuria / discoloration of the urine
Hepatomegaly in some cases

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3
Q

Expected lab findings in patients with hemolytic anemia:

Hemoglobin level?

A

Normal to severely reduced

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4
Q

Expected lab findings in patients with hemolytic anemia:

MCV MCH?

A

Increased MCV
Increased MCH

MCV - macrocytes on blood smear
MCH - polychromasia

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5
Q

Expected lab findings in patients with hemolytic anemia:

Reticulocytes?

A

Increased

Main sign of the erythropoietic response by the bone marrow

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6
Q

Expected lab findings in patients with hemolytic anemia:

Bilirubin?
LDH?
Haptoglobulin?

A

Bilirubin? Increased, unconjugated
(Also with increased AST with uronilinogen in urine and stool)

LDH? Increased up to 10x normal - especially in intravascular hemolysis

Haptoglobulin? Reduced to absent

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7
Q

The telltale sign that hemolysis is mainly intravascular?

A

Presence of hemoglobinuria or hemosiderinuria

Intravascular hemolysis - Hemoglobinuria - Iron loss - Need iron supplements

Extravascular hemolysis - need blood transfusions - iron overload - secondary hemochromatosis - damage to liver and heart

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8
Q

If hemolysis is recurring, increase in bilirubin favors formation of:

A

Gallstones

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9
Q

Situations where compensated hemolysis (presence of hemolysis with no anemia) becomes hemolytic anemia?

A

Pregnancy
Folate deficiency
Renal disease - ineffective EPO production

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10
Q

What virus causes aplastic anemia?

A

Parvovirus B19

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11
Q

What is the main diagnostic test for Hereditary Spherocytosis?

A

Osmotic fragility test - red cells were abnormally susceptible to lysis in hypotonic solution

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12
Q

Main triad of clinical findings in HS?

A
  1. Jaundice
  2. Enlarged spleen
  3. Gallstones
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13
Q

Only condition with increased MCHC on an ordinary blood count report?

A

Hereditary Spherocytosis

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14
Q

Dual mechanism of spleen in HS

A
  1. Major site of red cell destruction

2. Make defective red cells more spherocytic which accelerates lysis elsewhere

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15
Q

When is the proper time to do splenectomy in mild cases of HS?

A

Avoid splenectomy

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16
Q

When is the proper time to do splenectomy in moderate cases of HS?

A

Delay until puberty

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17
Q

When is the proper time to do splenectomy in severe cases of HS?

A

Delay until 4-6 years of age

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18
Q

What vaccination is imperative prior to splenectomy in HS?

A

Antipneumococcal vaccine

Prophylaxis with penicillin after splenectomy is controversial

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19
Q

Recommendations re splenectomy in HE (Hereditary Elliptocytosis)?

A

Splenectomy may be beneficial in all cases

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20
Q

Autosomal dominant disorder of cation transport that is characterized by increased intracellular sodium with concomitant loss of potassium?

A

Pseudohyperkalemia

Incidental finding of elevated serum K

21
Q

Disorder of cation transport associated with overhydrated red cells, with linear shaped central pallor in blood smear?
Expected MCHC?

A 
Stomatocytosis 
Low MCHC (overhydrated, gain water)
22
Q

Disorder of cation transport associated with dehydrated red cells, with resulting rigidity? Expected MCHC?

A

Xerocytosis

High MCHC

23
Q

Recommendations regarding splenectomy in patients with stomatocytosis?

A

Strongly contraindicated to do splenectomy, due to severe thromboembolic complications

24
Q

Hemolytic anemia that often presents as neonatal jaundice in newborns (the jaundice persists) and very high reticulocytosis?

A

Homozygous pyruvate kinase deficiency

PK mutation
Inherited and rare

25
Q

What compensatory mechanism in pyruvate kinase deficiency makes anemia well tolerated causing a delay in diagnosis?

A

Increase in bisphosphoglycerate (DPG)

Oxygen delivery to the tissue is enhanced

26
Q

Treatment of Pyruvate Kinase deficiency

A

Mainly supportive

  1. Folic acid supplements
  2. Blood transfusion + Iron chelation
  3. Splenectomy
  4. Marrow transplant from HLA identical, PK normal sibling
27
Q

G6PD Deficiency confers a relative resistance against what specific infection?

A

Plasmodium falciparum malaria

28
Q

Common presentation of G6PD Deficiency in newborns?

A

Neonatal Jaundice (NNJ)

Very rarely present at birth
Onset between day 2-3
Exacerbated by: Prematurity, infection, naphthalene balls

29
Q

Dreaded complication if NNJ in G6PD Deficiency is left untreated?

A

Kernicterus

Permanent neurologic damage

30
Q

Type of anemia in G6PD Deficiency?

A

Normocytic normochromic

Moderate to extremely severe
Jaundice
Hemoglobinuria
High LDH, low haptoglobin

31
Q

Typical feature of G6PD Deficiency are bizarre poikilocytes with red cells that appear to have unevenly distributed hemoglobin called ____ and red cells that have parts bitten away called ____

A

Hemighosts

Bite cells / Blister cells

32
Q

Test in G6PD Deficiency with methylviolet reveal precipitates of denatured hemoglobin and hemichromes called ____

A

Heinz bodies

Signature of oxidative damage to red cells

33
Q

Most serious threat of acute HA is:

A

Acute renal failure

But full recovery from G6PD Deficiency is expected

34
Q

Diagnostic test for G6PD Deficiency?

A

Quantitative tests for G6PD

Needed when the patient has had a hemolytic attack

35
Q

Prevention of AHA in G6PD Deficiency is done by avoiding triggers, which are:

A

Fava beans
Infections
Drugs

36
Q

Rare peculiar self limiting severe HA associated with deficiency of glutathione peroxidase?

A

Infantile poikilocytosis

Due to transient deficiency of selenium

37
Q

What red cell enzyme defect presents with a deficiency seen in red cells as basophilic stippling, anemia is lifelong and may benefit from splenectomy?

A

Pyrimidine 5’ Nucleotidase (P5N) Deficiency

38
Q

Differentiate Warm and Cold Immune Hemolytic Anemias in terms of:

Autoantibody?
Treatment?

A

Warm:
Autoantibody IgG
Treatment Splenectomy, Immunosuppression and Steroids

Cold:
Autoantibody IgM
Treatment Plasmapheresis, Cyclophosphamide or Azathioprine (Alkylating agents)

39
Q

Conditions with basophilic stippling

A

Lead poisoning

P5N Deficiency

40
Q

Condition associated with Bite cells in peripheral smear?

A

G6PD

41
Q

Condition with Heinz bodies after staining with methyl violet?

A

G6PD Deficiency

42
Q

PNH is a rare clonal stem cell disorder caused by a defect in expression of RBC membrane proteins called:

A

CD55 CD59

43
Q

Previously the best test to diagnose PNH before the development of assays for CD55 CD59

A

Ham’s test (sucrose hemolysis test)

Gold standard today: Flow cytometry to check for levels of CD55 CD59

44
Q

Classic triad of PNH

A

Hemolysis (intravascular)
Pancytopenia
Thrombosis (venous)

45
Q

When venous thrombosis is severe in PNH, hepatic veins may be affected leading to the development of what syndrome

A

Budd Chiari Syndrome

Acute hepatomegaly with ascites

46
Q

Treatment of PNH

A

Folic acid supplement
Transfusion of filtered red cells
Iron supplement as appropriate

*Glucocorticoids are contraindicated

47
Q

Main mechanism of PNH causing intravascular hemolysis

A

Complement mediated destruction of CD59 Red cells

48
Q

Main mechanism for intravascular hemolysis of Septicemia

A

Exotoxins produced by Clostridium perfringens

49
Q

Main mechanism for intravascular hemolysis of Microangiopathic anemia, usually found in patients with leaky prosthetic heart valves

A

Red cell fragmentation - check red cell morphology on blood smear

Hematology (9 decks)

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