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Date of last update: 10/12/2017.

Forum Name: Hematology Topics

Question: treatment of fulmenent autoimmune hemolytic anemia

 anisa - Wed Aug 04, 2010 4:46 pm

My mother 51 years started to complain of general weakness and paller red hemoglobin before one weak her Hb was 4.5 g/dl and direct coombs test was positive, she admitted to hospital as autoimmune hemolytic anemia and 2 pints of whole blood transfused with 60mg oral prednisolone, yesterday her Hb drop to 3.2 g/dl with yallow eye and red urine and the doctor change oral prednisolone to 80 mg metylprednisolone, to day her Hb is 2.9 g/dl.
She is in fulmenent hemolysis as Dr said please I need your urgent help.
 Dr.M.Aroon kamath - Fri Aug 06, 2010 12:45 pm

User avatar Hi,
I empathize with you and fully understand your genuine concerns. An understanding of the autoimmune hemolytic anemias may help you in comprehending the situation better.

Autoimmune hemolytic anemia(AIHA) may be classified as,

- Warm antibody hemolytic anemia: is the most common form of AIHA. Autoantibodies in in this condition generally react at temperatures ≥ 37°C. Most of the autoantibodies are of the IgG class. This condition may be idiopathic, present in association with certain other diseases such as lymphoma,Systemic lupus erythematosus,or chronic lymphocytic leukemia) or in some cases are drug induced.
- cold antibody disease: is caused by autoantibodies that react at temperatures < 37°C. Autoantibodies are usually IgM. Infections tend to cause acute hemolytic disease.

Paroxysmal cold hemoglobinuria (PCA): is a subtype of cold agglutinin disease which is rare. Hemolysis results from exposure to cold (generalized or localized). Activities such as drinking cold liquids, washing hands in cold water may precipitate acute hemolysis. An IgG autohemolysin is involved in the pathogenesis. This binds to RBCs at low temperatures and causes intravascular hemolysis upon re-warming(as the body temperature reverts to normal). It occurs most often following a nonspecific viral illness.

Most patients who have AIHA have mild anemia of limited duration and, therefore, do not need therapy. Because most of the chronic cases are mild, minimal or no intervention is necessary.

Following are the management modalities that may be needed (singly or in combination).

Blood transfusion: Blood transfusion is needed only in life-threatening situations or as a interim measure while waiting for other tratment modalities to take effect.

Steroids: About 80% of patients who have idiopathic warm antibody hemolytic anemia, respond to steroids (1 to 2 mg/kg per day).

Intravenous Immune Globulin (IVIG): IVIG is effective in doses of 1 to 2 g/kg administered over 1 to 2 days.May be useful in the management of drug induced warm antibody hemolytic anemia after withdrawal of the offending drug.

Splenectomy: Splenectomy is effective in cases of AIHA in which most of the hemolysis is in the spleen, such as IgG-induced AIHA. It is not as effective in IgM-induced disease because most of the hemolysis in this condition occurs intravascularly. In PCA splenectomy has no role.

Coomb's test only diagnoses the cause of hemolysis as AIHA.

There are 2 types of Coombs Reagents available.
- Polyspecific reagent: Containing both anti IgG and antiC3,
- monospecific reagent: Either containing anti IgG or anti C3.

Thus it is possible to identify the presence of complement 3 (C3) separately from IgG.

With these results in hand, one may be able to diagnose the subtypes as follows:
- positive with anti-IgG and negative with anti-C3: probably, idiopathic warm antibody AIHA or the drug-associated AIHA.
- positive with anti-IgG and anti-C3: SLE associated case, idiopathic warm antibody AIHA, or rarely drug-associated AIHA.

I sincerely hope your mother is better by now. Hope this information is useful in some way.
Best wishes!

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