Histiocytic medullary reticulosis was first described as a clinical and pathological entity by Scott and Robb-Smith in 1939.
The clinical features include fever, malaise, weakness, weight loss, lymphadenopathy, hepatosplenomegaly, progressive pancytopenia, jaundice, purpura and occasionally popular skin eruptions or tumors. The outstanding pathological features of the disease are proliferation of hitiocytes throughout the lymphoreticular system and frequent erythrophagocytosis.
The clinical course is acute, rapidly fatal and average duration of survival before death is 15 weeks. The treatment is usually of no avail. The case, reported here, was unusual in that the patient manifested specific skin lesions, the clinical presumptive diagnosis was confirmed by the histologic findings of skin lesion biopsy and a bone-marrow aspiration and in that the patient had a objective remission induced combination chemotherapy with prednisolone, vincristine and endoxan.
The patient eventually died without significant prolongation of survival time. A brief review of the literature was presented.
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