HEMATOLOGICAL DISORDERS AS THE FIRST MANI FESTATIONS OF SARCOIDOSIS WITH HYPOGAMMAGLOBULINEMIA

Background. - Hematological changes are uncommon in childhood sarcoido sis. Case report. - Case 1. This patient developed severe hemolytic an emia associated with splenomegaly at the age of 11 months. A second ep isode of hemolysis was seen at the age of 6 years; it was associated w ith thrombocytopenia and hypogammaglobulinemia. A partial splenectomy permitted histological diagnosis of sarcoidosis that was confirmed by elevated angiotensin-converting enzyme level, and subsequently develop ment of pulmonary involvement. Case 2. This boy differed from autoimmu ne hemolytic anemia and thrombocytopenia associated with splenomegaly at the age of 3 years. At the age of 7 years, he had splenomegaly, enl arged cervical and lomboaortic lymph nodes and hypogammaglobulinemia. Diagnosis of sarcoidosis was made from lymph nodes and spleen biopsies after splenectomy; the patient had increased angiotensin-converting e nzyme concentration, and subsequently developed specific renal involve ment. Conclusion. - Association of hemolytic anemia, thrombocytopenia, hypogammaglobulinemia and splenomegaly should suggest the diagnosis o f sarcoidosis and could be considered as part of the disease spectrum.