Chapter 129 Hemolytic Anemia And Anemia due to Acute Blood Loss

Question 1

Main sign in patients with hemolytic anemia?

Answer 1

Jaundice

Other signs:
Pallor

Question 2

Main symptom of patients with hemolytic anemia ?

Answer 2

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

Question 3

Expected lab findings in patients with hemolytic anemia:

Hemoglobin level?

Answer 3

Normal to severely reduced

Question 4

Expected lab findings in patients with hemolytic anemia:

MCV MCH?

Answer 4

Increased MCV
Increased MCH

MCV - macrocytes on blood smear
MCH - polychromasia

Question 5

Expected lab findings in patients with hemolytic anemia:

Reticulocytes?

Answer 5

Increased

Main sign of the erythropoietic response by the bone marrow

Question 6

Expected lab findings in patients with hemolytic anemia:

Bilirubin?
LDH?
Haptoglobulin?

Answer 6

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

Question 7

The telltale sign that hemolysis is mainly intravascular?

Answer 7

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

Question 8

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

Answer 8

Gallstones

Question 9

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

Answer 9

Pregnancy
Folate deficiency
Renal disease - ineffective EPO production

Question 10

What virus causes aplastic anemia?

Answer 10

Parvovirus B19

Question 11

What is the main diagnostic test for Hereditary Spherocytosis?

Answer 11

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

Question 12

Main triad of clinical findings in HS?

Answer 12

1. Jaundice
2. Enlarged spleen
3. Gallstones

Question 13

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

Answer 13

Hereditary Spherocytosis

Question 14

Dual mechanism of spleen in HS

Answer 14

1. Major site of red cell destruction

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

Question 15

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

Answer 15

Avoid splenectomy

Question 16

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

Answer 16

Delay until puberty

Question 17

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

Answer 17

Delay until 4-6 years of age

Question 18

What vaccination is imperative prior to splenectomy in HS?

Answer 18

Antipneumococcal vaccine

Prophylaxis with penicillin after splenectomy is controversial

Question 19

Recommendations re splenectomy in HE (Hereditary Elliptocytosis)?

Answer 19

Splenectomy may be beneficial in all cases

Question 20

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

Answer 20

Pseudohyperkalemia

Incidental finding of elevated serum K

Question 21

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

Answer 21

Stomatocytosis 
Low MCHC (overhydrated, gain water)

Question 22

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

Answer 22

Xerocytosis

High MCHC

Question 23

Recommendations regarding splenectomy in patients with stomatocytosis?

Answer 23

Strongly contraindicated to do splenectomy, due to severe thromboembolic complications

Question 24

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

Answer 24

Homozygous pyruvate kinase deficiency

PK mutation
Inherited and rare

Question 25

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

Answer 25

Increase in bisphosphoglycerate (DPG) Oxygen delivery to the tissue is enhanced

Question 26

Treatment of Pyruvate Kinase deficiency

Answer 26

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

Question 27

G6PD Deficiency confers a relative resistance against what specific infection?

Answer 27

Plasmodium falciparum malaria

Question 28

Common presentation of G6PD Deficiency in newborns?

Answer 28

Neonatal Jaundice (NNJ) Very rarely present at birth Onset between day 2-3 Exacerbated by: Prematurity, infection, naphthalene balls

Question 29

Dreaded complication if NNJ in G6PD Deficiency is left untreated?

Answer 29

Kernicterus | Permanent neurologic damage

Question 30

Type of anemia in G6PD Deficiency?

Answer 30

Normocytic normochromic Moderate to extremely severe Jaundice Hemoglobinuria High LDH, low haptoglobin

Question 31

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 ____

Answer 31

Hemighosts | Bite cells / Blister cells

Question 32

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

Answer 32

Heinz bodies Signature of oxidative damage to red cells

Question 33

Most serious threat of acute HA is:

Answer 33

Acute renal failure But full recovery from G6PD Deficiency is expected

Question 34

Diagnostic test for G6PD Deficiency?

Answer 34

Quantitative tests for G6PD Needed when the patient has had a hemolytic attack

Question 35

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

Answer 35

Fava beans Infections Drugs

Question 36

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

Answer 36

Infantile poikilocytosis Due to transient deficiency of selenium

Question 37

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

Answer 37

Pyrimidine 5' Nucleotidase (P5N) Deficiency

Question 38

Differentiate Warm and Cold Immune Hemolytic Anemias in terms of: Autoantibody? Treatment?

Answer 38

Warm: Autoantibody IgG Treatment Splenectomy, Immunosuppression and Steroids Cold: Autoantibody IgM Treatment Plasmapheresis, Cyclophosphamide or Azathioprine (Alkylating agents)

Question 39

Conditions with basophilic stippling

Answer 39

Lead poisoning | P5N Deficiency

Question 40

Condition associated with Bite cells in peripheral smear?

Answer 40

G6PD

Question 41

Condition with Heinz bodies after staining with methyl violet?

Answer 41

G6PD Deficiency

Question 42

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

Answer 42

CD55 CD59

Question 43

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

Answer 43

Ham's test (sucrose hemolysis test) Gold standard today: Flow cytometry to check for levels of CD55 CD59

Question 44

Classic triad of PNH

Answer 44

Hemolysis (intravascular) Pancytopenia Thrombosis (venous)

Question 45

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

Answer 45

Budd Chiari Syndrome Acute hepatomegaly with ascites

Question 46

Treatment of PNH

Answer 46

Folic acid supplement Transfusion of filtered red cells Iron supplement as appropriate *Glucocorticoids are contraindicated

Question 47

Main mechanism of PNH causing intravascular hemolysis

Answer 47

Complement mediated destruction of CD59 Red cells

Question 48

Main mechanism for intravascular hemolysis of Septicemia

Answer 48

Exotoxins produced by Clostridium perfringens

Question 49

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

Answer 49

Red cell fragmentation - check red cell morphology on blood smear