Abstract
Autoimmune hemolytic anemia (AIHA) is a heterogeneous disease usually classified as warm, cold [cold agglutinin disease (CAD)] or mixed, according to the thermal range of the autoantibody. Diagnosis is based on the direct antiglobulin test (DAT), typically positive with anti-IgG antisera in warm AIHA and anti-C3d in CAD. Diagnostic pitfalls are due to IgA autoantibodies, warm IgM, low-affinity IgG, or IgG below the threshold of sensitivity, and about 5% of AIHA remains DAT-negative. The treatment of AIHA is still not evidence-based. Corticosteroids are the first-line therapy for warm AIHA. For refractory/relapsed cases, the choice is between splenectomy (effective in ~70% cases but with a presumed cure rate of 20%) and rituximab (effective in ~70%-80% of cases), which is becoming the preferred second-line treatment, and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil). Rituximab is now recommended as first-line treatment for CAD. Additional therapies are intravenous immunoglobulins, danazol, and plasma exchange, with alemtuzumab and high-dose cyclophosphamide as the last options.
| Original language | English |
|---|---|
| Pages (from-to) | 304-312 |
| Number of pages | 9 |
| Journal | Seminars in Hematology |
| Volume | 52 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - Oct 1 2015 |
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ASJC Scopus subject areas
- Hematology
Cite this
Immune Hemolysis : Diagnosis and Treatment Recommendations. / Barcellini, Wilma.
In: Seminars in Hematology, Vol. 52, No. 4, 01.10.2015, p. 304-312.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Immune Hemolysis
T2 - Diagnosis and Treatment Recommendations
AU - Barcellini, Wilma
PY - 2015/10/1
Y1 - 2015/10/1
N2 - Autoimmune hemolytic anemia (AIHA) is a heterogeneous disease usually classified as warm, cold [cold agglutinin disease (CAD)] or mixed, according to the thermal range of the autoantibody. Diagnosis is based on the direct antiglobulin test (DAT), typically positive with anti-IgG antisera in warm AIHA and anti-C3d in CAD. Diagnostic pitfalls are due to IgA autoantibodies, warm IgM, low-affinity IgG, or IgG below the threshold of sensitivity, and about 5% of AIHA remains DAT-negative. The treatment of AIHA is still not evidence-based. Corticosteroids are the first-line therapy for warm AIHA. For refractory/relapsed cases, the choice is between splenectomy (effective in ~70% cases but with a presumed cure rate of 20%) and rituximab (effective in ~70%-80% of cases), which is becoming the preferred second-line treatment, and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil). Rituximab is now recommended as first-line treatment for CAD. Additional therapies are intravenous immunoglobulins, danazol, and plasma exchange, with alemtuzumab and high-dose cyclophosphamide as the last options.
AB - Autoimmune hemolytic anemia (AIHA) is a heterogeneous disease usually classified as warm, cold [cold agglutinin disease (CAD)] or mixed, according to the thermal range of the autoantibody. Diagnosis is based on the direct antiglobulin test (DAT), typically positive with anti-IgG antisera in warm AIHA and anti-C3d in CAD. Diagnostic pitfalls are due to IgA autoantibodies, warm IgM, low-affinity IgG, or IgG below the threshold of sensitivity, and about 5% of AIHA remains DAT-negative. The treatment of AIHA is still not evidence-based. Corticosteroids are the first-line therapy for warm AIHA. For refractory/relapsed cases, the choice is between splenectomy (effective in ~70% cases but with a presumed cure rate of 20%) and rituximab (effective in ~70%-80% of cases), which is becoming the preferred second-line treatment, and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil). Rituximab is now recommended as first-line treatment for CAD. Additional therapies are intravenous immunoglobulins, danazol, and plasma exchange, with alemtuzumab and high-dose cyclophosphamide as the last options.
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UR - http://www.scopus.com/inward/citedby.url?scp=84941917679&partnerID=8YFLogxK
U2 - 10.1053/j.seminhematol.2015.05.001
DO - 10.1053/j.seminhematol.2015.05.001
M3 - Article
C2 - 26404442
AN - SCOPUS:84941917679
VL - 52
SP - 304
EP - 312
JO - Seminars in Hematology
JF - Seminars in Hematology
SN - 0037-1963
IS - 4
ER -