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Chapter 60: Autoimmune Hemolytic Anemia

James N. Frame

INTRODUCTION

Autoimmune hemolytic anemia (AIHA) is an acquired immunologic disease in which a patient's red blood cells are selectively destroyed or hemolyzed by autoantibodies (auto-Ab) produced by the patient's own immune system

 

Mack P, Freedman J. Transfus Med Rev 2000;14: 223–233

 

Etiology

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Etiology
  Percent of Cases
1. Idiopathic 58%    
2. Secondary 42%    
 Neoplasia   18%  
  Carcinomas     25%
  CLL     22%
  NHL     18%
  MDS     9%
  Hodgkin disease     5%
 Drug-induced   8%  
  Other immune based/or miscellaneous   7%  
 Infection   5%  
  Unspecified     38%
   Mycoplasma pneumoniae     33%
   Viral/unspecified pneumonia     17%
  Mononucleosis     7%
  Collagen vascular diseases   4%  
   Rheumatoid arthritis     61%
   Systemic lupus erythematosus     37%

Gehrs BC, Friedberg RC. Am J Hematol 2002;69: 258–271

Pirofsky B. Semin Hematol 1976;13:251–265

Sokol RJ et al. J Clin Pathol 1992;45:1047–1052

Epidemiology

  • Idiopathic AIHA is more common in women than men (2:1) with a peak incidence in the fourth and fifth decades of life. Idiopathic AIHA is not associated with any demonstrable underlying disease

  • Secondary causes have been reported in 20–80% of case series

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Epidemiology
  United Kingdom✫ United States
Incidence/Year Percent of Cases Percent of Cases
Overall 1/25,600 — —
Warm antibody AIHA 1/40,800 63 48–78
Cold antibody AIHA 1/87,500 29 16–32
Paroxysmal cold hemoglobinuria (PCH) 1/2,470,000 1 7–8
Mixed-type AIHA 1/370,000 7 3

✫Data from a 10-year study (1982–1991) of 1834 patients evaluated for suspected AIHA or because of RBC interference in blood grouping or typing from a 4.7 million population base at the Regional Blood Transfusion Center, Sheffield, UK (Sokol RJ et al. J Clin Pathol 1992;45:1047–1052)
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Epidemiology

Spectrum of Drug-Induced Immune Hemolytic Anemia (DIIHA)
Variable Johnson ST et al. (2007) Garratty G et al. (2003)
Retrospective study period, number of patients

20-year experience, 71 patients with 73 drug-dependent antibodies

Blood Center of Wisconsin, Inc

Milwaukee, WI, USA✫

25-year experience (1978–2003), 114

American Red Cross Blood Services

Los Angeles, CA, USA†

Agents, number (%)

 Cephalosporins

 Cefotetan/ceftriaxone

Penicillin and/or derivatives

Piperacillin

 Piperacillin + tazobactam

 Tazobactam

 Clavulanate

 Sulbactam

 Ticarcillin

 Ampicillin

 Nafcillin

NSAIDs (various)

Quinine/quinidine

Others

 

37 (51)

27 (37)/5 (7)

12 (16)

5

5

—

—

—

—

1

1

11 (15)

7;6/1 (10)

6 (8); 1 each: carboplatin, levofloxacin, cefoxitin, oxaliplatin, probenecid

 

84 (74)

72 (63)/10 (9)

17 (15)

4

—

5

3

1

1

—

1

—

—

13 (11); 2 each: fludarabine, probenecid, rifampin; 1 each: chlorpropamide, erythromycin, mefloquine, oxaliplatin, phenacetin, procainamide, tolmetin

(+) DAT results, number (%)

IgG + C3

IgG only

C3 only

 

46/68 (68)

17/68 (25)

5/68 (7)

 

81/113 (70)

11/113 (10)

11/113 ...

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