Autoimmune hemolytic anemia: Difference between revisions

Latest revision as of 11:16, 29 July 2020

Differential Diagnosis

Cold AIHA
- Primary: cold agglutinin disease (CAD)
- Secondary
  - Viral infections: EBV, CMV, others
  - Other infections: Mycoplasma (paroxysmal cold hemoglobinuria)
  - Other diseases: Waldenström macroglobulinema, lymphoma
  - Drugs: lenalidomide
Warm AIHA
- Primary: warm AIHA
- Secondary
  - Viral infections: HIV, vaccines
  - Other diseases: lupus, CLL and other malignancies, chronic inflammatory disorders (including IBD)
  - Allogeneic hematopoietic stem cell transplantation
  - Drugs: cefotetan, ceftriaxone, piperacillin, immune checkpoint-modulating drugs (e.g. nivolumab)
Drug-related
- Penicillin
- Methyldopa

Management

Warm AIHA

Can be an emergency if severe and acute
If life-threatening, transfuse ABO-matched but incompatible blood, thereby sparing the patient's own cells
First-line: prednisone 1 mg/kg daily (response in 50% of patients)
Second-line: rituximab 100 mg/week for 4 weeks, with prednisone
Refractory: splenectomy

Cold agglutinin disease

Chronic hemolysis in older patients caused by IgM antibodies produced by a B-lymphocyte clone (can show up as MGUS), therefore is essentially a form of Waldenström macroglobulinema
First-line: rituximab ± fludarabine
Prednisone and splenectomy are not helpful

@@ Line 1: / Line 1: @@
+==Differential Diagnosis==
-== Etiologies ==
-* Cold AIHA
+*Cold AIHA
-** Primary: cold agglutinin disease (CAD)
+**Primary: [[cold agglutinin disease]] (CAD)
-** Secondary
+**Secondary
-*** Viral infections: EBV, CMV, others
+***Viral infections: [[EBV]], [[CMV]], others
-*** Other infections: Mycoplasma (paroxysmal cold hemoglobinuria)
+***Other infections: [[Mycoplasma]] (paroxysmal cold hemoglobinuria)
-*** Other diseases: Waldenström macroglobulinema, lymphoma
+***Other diseases: [[Waldenström macroglobulinema]], [[lymphoma]]
-*** Drugs: lenalidomide
+***Drugs: [[lenalidomide]]
-* Warm AIHA
+*Warm AIHA
-** Primary: warm AIHA
+**Primary: warm AIHA
-** Secondary
+**Secondary
-*** Viral infections: HIV, vaccines
+***Viral infections: [[HIV]], vaccines
-*** Other diseases: lupus, CLL and other malignancies, chronic inflammatory disorders (including IBD)
+***Other diseases: [[lupus]], [[CLL]] and other malignancies, chronic inflammatory disorders (including [[IBD]])
-*** Allogeneic stem cell transplant
+***Allogeneic [[hematopoietic stem cell transplantation]]
-*** Drugs: cefotetan, ceftriaxone, piperacillin, immune checkpoint-modulating drugs (e.g. nivolumab)
+***Drugs: [[cefotetan]], [[ceftriaxone]], [[piperacillin]], immune checkpoint-modulating drugs (e.g. [[nivolumab]])
-* Drug-related
+*Drug-related
-** Penicillin
+**[[Penicillin]]
-** Methyldopa
+**[[Methyldopa]]
-== Management ==
+==Management==
-=== Warm AIHA ===
+===Warm AIHA===
-* Can be an emergency if severe and acute
+*Can be an emergency if severe and acute
-* If life-threatening, transfuse ABO-matched but incompatible blood, thereby sparing the patient's own cells
+*If life-threatening, transfuse ABO-matched but incompatible blood, thereby sparing the patient's own cells
-* First-line: prednisone 1 mg/kg daily (response in 50% of patients)
+*First-line: [[prednisone]] 1 mg/kg daily (response in 50% of patients)
-* Second-line: rituximab 100 mg/week for 4 weeks, with prednisone
+*Second-line: [[rituximab]] 100 mg/week for 4 weeks, with prednisone
-* Refractory: splenectomy
+*Refractory: [[splenectomy]]
-=== Cold agglutinin disease ===
+===Cold agglutinin disease===
-* Chronic hemolysis in older patients caused by IgM antibodies produced by a B-lyphocyte clone (can show up as MGUS), therefore is essentially a form of Waldenström macroglobulinema
+*Chronic hemolysis in older patients caused by IgM antibodies produced by a B-lymphocyte clone (can show up as MGUS), therefore is essentially a form of [[Waldenström macroglobulinema]]
-* First-line: Rituximab ± fludarabine
+*First-line: [[rituximab]] ± [[fludarabine]]
-* Prednisone and splenectomy are ''not'' helpful
+*[[Prednisone]] and [[splenectomy]] are ''not'' helpful
 [[Category:Hematology]]