A Case of Pure Red Cell Aplasia Associated with Sinus Histiocytosis with Multiple Lymphadenopathy / 대한혈액학회지

ABSTRACT

Pure red cell aplasia (PRCA) is characterized by severe anemia with reticulocytopenia and absence of erythroblast from the bone marrow. Sinus histiocytosis with massive lymphadenopathy (SHML Rosai-Dorfman disease) is rare systemic disease characterized by painless cervical, axillary, inguinal and mediastinal lymphadenopathy and frequent extranodal invasion. Histologically, lymph node sinuses are expanded by numerous distinctive histiocytes which contains well preserved lymphocytes. We report a case of sinus histiocytosis with massive lymphadenopathy in inguinal lymph node biopsy with polyclonal gammopathy and pure red cell aplasia in bone marrow biopsy who was infected by Epstein-Barr virus.