Dr Sarath Menon presents an approach to diagnosing and classifying hemolytic anemia. Hemolytic anemia results from increased red blood cell destruction and bone marrow compensation. It can be congenital/hereditary or acquired. Classification includes intracorpuscular defects like hemoglobinopathies and enzymopathies, and extracorpuscular factors like mechanical destruction, toxic agents, infections, and autoimmune causes. Diagnosis involves confirming hemolysis and determining the etiology through history, physical exam, peripheral smear, and ancillary lab tests. Common etiologies discussed in detail include sickle cell disease, thalassemia, G6PD deficiency, membrane defects like hereditary spherocytosis, and autoimmune
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Approach to Diagnosing and Classifying Hemolytic Anemia
1. APPROACH TO HEMOLYTIC ANEMIA
Candidate: Dr SARATH MENON.R
K.B.ILLAVA HEMATOLOGY DIVISION
DEPT.MEDICINE,
MGM MEDICAL COLLEGE,INDORE
2. OBJECTIVES
Lab indication of hemolysis
Intravascular v/s extravascular hemolysis
D/D of hemolytic anemia
Diagnose hemo.anemia with peripheral smear &
ancillary lab tests
3. HEMOLYTIC ANEMIA
Definition:
Those anemias which result from an increase in RBC
destruction coupled with increased erythropoiesis
Classification:
Congenital / Hereditary
Acquired
12. THE KEY TO THE ETIOLOGY OF
HEMOLYTIC ANEMIA
The history
The peripheral blood film
13. PATIENT HISTORY
Acute or chronic
Medication/Drug precipitants
G6PD
AIHA
Family history
Concomitant medical illnesses
Clinical presentation
14. CASE 1
3 yr old male child presenting with pallor,jaundice,
Severe pain of long bones, fever
CBC-anemia,reticulocytosis,increased WBC
LAB - LDH -600 (normal upto 200)
S.bilirubin- 5mg%
27. BETA THALASSEMIA MAJOR
Severe homozygous
Childhood, growth delay
Severe anemia,hepatosplenomegaly,r/r transfusion
Iron overload-endo.dysfnct
P.Smear- severe microcytosis,target cells
Hb electro- HbF - 90-96 %
HbA2- 3.5 %- 5.5%
HbA - 0 %
28. BETA THALASSEMIA INTERMEDIA
Similar stigmata like major
Survive without c/c transfusion
Less severe than major
Moderate anemia,microcytosis,hypochromia
Hb electrophor- HbF - 20-100%
HbA2 -3.5%-5.5%
HbA – 0-30%
29. BETA THALASSEMIA MINOR
Profound microcytosis,target cells
Minimal anemia
Similar bld picture of iron def.anemia
Lab inv:
MCV<75,Hct <30-33%
Hb electr: HbA2-3.5-7.5%,HbA-80-95%,HbF-1-5%
31. CASE 3
45 yr old male came to opd in a remote PHC with
burning micturition
Urine R/M shows numerous pus cells++++
UTI diagnosed & medical officer gave
cotrimoxazole 2 bd X 5days
1 wk later,pt developed severe
pallor,palpitation,jaundice
Lab- increased LDH, S.BILIRUBIN,RETIC COUNT
P.S- shows irreg cells like
42. RED CELL MEMBRANE DEFECTS
1.Hereditary Spherocytosis
Usually inherited as AD disorder
Defect: Deficiency of Beta Spectrin or Ankyrin Loss of
membrane surface area becomes more spherical
Destruction in Spleen
44. COMPLICATIONS
Clinical course may be complicated with Crisis:
Hemolytic Crisis: associated with infection
Aplastic crisis: associated with Parvovirus infection
45. Inv:
Test will confirm Hemolysis
P Smear: Spherocytes
Osmotic Fragility: Increased
Screen family members
46. AUTOIMMUNE HEMOLYTIC ANEMIA
Result from RBC destruction due to RBC
autoantibodies: Ig G, M, E, A
Most commonly-idiopathic
Classification
Warm AI hemolysis:Ab binds at 37degree Celsius
Cold AI Hemolysis: Ab binds at 4 degree Celsius
47. 1.Warm AI Hemolysis:
Can occurs at all age groups
F > M
Causes:
50% Idiopathic
Rest - secondary causes:
1.Lymphoid neoplasm: CLL, Lymphoma,
Myeloma
2.Solid Tumors: Lung, Colon, Kidney, Ovary,
Thymoma
3.CTD: SLE,RA
4.Drugs: Alpha methyl DOPA, Penicillin ,
Quinine, Chloroquine
5. UC, HIV
48. Inv:
hemolysis, MCV decreased
P Smear: microspherocytosis,
Confirmation: Direct Coomb’s Test / Antiglobulin test
49.
50. • 2. Cold AI Hemolysis
Usually Ig M directed at the RBC I antigen
Infection: Mycoplasma pneumonia, Infec Mononucleosis
Neoplasms : waldenstrom macroglobulinemia ,
lymphoma,CLL,kaposi sarcoma, myeloma.
C/F:
Elderly patients
Exacerbations in the winter
Cold , painful & often blue fingers, toes,
ears, or nose ( Acrocyanosis)
51. Inv:
e/o hemolysis
P Smear: Microspherocytosis
DAT positive with polyspecific and anticompliment antisera
52. CASE 5
32 yr old presented 4 days history of distention of
abdomen and rt hypochondrial pain and has h/o
passage of dark colored urine at night for weeks
On USG- hepatomegaly,gross ascites,hepatic vein
thrombosis
Lab : Hb – 7gm%. WBC- 2200, PLC- 80,000
LDH- 600, S.BR- 4 mg%
urine bile pigment +,heme dip stick++
What is the diagnosis?
53.
54. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
Acquired chronic H.A
Persistent intra vascular hemolysis
Pancytopenia
Lab :hburia,hemosiderinuria,increased LDH,bilirubin
Risk of venous thrombosis
C/F – hemoglobinuria during night
P.S – polychromatophilia, normoblasts
B.M – normoblastic hyperplasia
Def.diagnosis-flow cytometry CD59-,CD55- RBC,WBC
- Hams’ acidified serum test
55. CASE 6
25 yr old male with RHD – severe MR done MVR,after 10
days presented with pallor, palpitation,jaundice
CBC shows Hb – 7.5 gm %, Hct -22 %
Lab : S.bilirubin -4.5mg%
LDH -600
Retic count 10%
Peripheral smear –
62. CONCLUSION
Hemolytic anemia can be recogised by clinical
picture-
- history & physical
- lab test to confirm hemolysis
- peripheral smear to guide further
tests