Treatment
Initial treatment was packed red blood cells & platelet transfusions for the pancytopaenia. He was then given intravenous immunoglobulin (IVIG) to create a dilutional effect and high pulse dose pulse of methylprednisolone and oral prednisone.
The seizures were treated with phenobarbitone.
Gradually the cytopaenias responded to therapy with resolution of the anaemia and normalisation of platelet count.
Post transfusion and glucocorticoid blood smears showed resolution of fragments and spherocytes.
Discharge blood results:
- Hb:11,8
- MCV: 79,5
- Platelets: 181
- Neutrophil: 3.8
- Anti Ro: 18
- Anti La: negative
Note: There are no established guidelines or consensus for effective treatment and management of this haematologic disease.
General recommendations are to try combinations or isolated forms of the following:
- Glucococorticoids
- IVIG
- Plasmapharesis
- Plasma exchange
