Table 1.

Investigations in patients presenting with AIHA

Diagnostic testsIndication for tests
Primary evaluation  
 Hemolytic screen  
  FBC, blood smear, LDH, haptoglobin, bilirubin, DAT, reticulocyte count with or without urine for hemosiderin or urine dipstick for microscopy  
 Detection of underlying disorders (investigation of AIHA)  
  Serum Igs and electrophoresis with immunofixation*  
  HIV, HBV, HCV  
  Anti-dsDNA, ANA  
  CT chest, abdomen, and pelvis  
Additional investigation in selected patients with AIHA  
 Bone marrow examination CAD, age ≥ 60 years, features in history, examination, FBC or smear suggesting possible marrow infiltration 
 U&E, LFT, clotting, BP, urine dipstick If pregnant or thrombocytopenic, to exclude DIC or pregnancy-associated TMA 
 Infection screening Dependent on symptoms, travel history, and age 
 Peripheral T-cell subsets, creatinine, LFT, clotting All children and if suspected Evans syndrome 
 Parvovirus, hematinics If reticulocytopenia 
Additional serological investigation in selected patients with AIHA  
 DAggT If DAT positive for C3d ± IgG 
 Cold antibody titer If DAggT positive 
 Monospecific DAT for IgM, G, A, C3d If DAT-negative AIHA suspected 
 Red cell eluate If (monospecific) DAT-negative AIHA suspected 
 Donath Landsteiner If DAT is positive for C3d ± IgG and 
i) DAggT-negative or insignificant CAs and 
ii) Age < 18 y or hemoglobinuria or cold-associated symptoms or atypical serology 
 Thermal range of cold antibody If clinical significance of cold autoagglutinin unclear 
Diagnostic testsIndication for tests
Primary evaluation  
 Hemolytic screen  
  FBC, blood smear, LDH, haptoglobin, bilirubin, DAT, reticulocyte count with or without urine for hemosiderin or urine dipstick for microscopy  
 Detection of underlying disorders (investigation of AIHA)  
  Serum Igs and electrophoresis with immunofixation*  
  HIV, HBV, HCV  
  Anti-dsDNA, ANA  
  CT chest, abdomen, and pelvis  
Additional investigation in selected patients with AIHA  
 Bone marrow examination CAD, age ≥ 60 years, features in history, examination, FBC or smear suggesting possible marrow infiltration 
 U&E, LFT, clotting, BP, urine dipstick If pregnant or thrombocytopenic, to exclude DIC or pregnancy-associated TMA 
 Infection screening Dependent on symptoms, travel history, and age 
 Peripheral T-cell subsets, creatinine, LFT, clotting All children and if suspected Evans syndrome 
 Parvovirus, hematinics If reticulocytopenia 
Additional serological investigation in selected patients with AIHA  
 DAggT If DAT positive for C3d ± IgG 
 Cold antibody titer If DAggT positive 
 Monospecific DAT for IgM, G, A, C3d If DAT-negative AIHA suspected 
 Red cell eluate If (monospecific) DAT-negative AIHA suspected 
 Donath Landsteiner If DAT is positive for C3d ± IgG and 
i) DAggT-negative or insignificant CAs and 
ii) Age < 18 y or hemoglobinuria or cold-associated symptoms or atypical serology 
 Thermal range of cold antibody If clinical significance of cold autoagglutinin unclear 

ANA, antinuclear antibody; BP, blood pressure; CAD, cold agglutinin disease; CAs, cold agglutinins; CT, computerized tomography; DAggT, direct agglutination test; DIC, disseminated intravascular coagulation; dsDNA, double-stranded DNA; FBC, full blood count; HBV, hepatitis B virus; HCV, hepatitis C virus; LFT, liver function tests; TMA, thrombotic microangiopathy; U&E, urea and electrolytes.

*

If a cold autoantibody suspected, keep sample at 37°C until serum has been separated.

Modified from Hill et al, with permission.12 

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