Histiocytic Medullary Reticulosis in Children: Report of Four Cases and Analysis of Previously Reported Cases

ABSTRACT

Histiocytic Medullary Reticulosis(H.M.R.) is a rapidly progressing fatal disease seen most often in adults, but it has been reported in children younger than 15 years of age. H.M.R. is clinically characterized by fever, wasting generalized lymphadenopathy and hepatosplenomegaly. In the terminal stage, jaundice, purpura, anemia and pancytopenia are all present with or without skin involvement. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the lymphoreticular tissues. Four cases of H.M.R. occurring in pediatric age group, i.e., 5 years, 6 years, 12 years and 14 years of age, respectively, are presented with discussion of the clinico-pathologic characteristics and management. Cases 1,2 and 3 died within 6 months after the onset of illness. Case 1 of our series was particularly younger than in previously reported cases. This was the case that the diagnosis was confirmed by postmortem examination. Analysis of previously reported 25 cases occurring in ages younger than 20 years of age was done.