Open AccessAN AUTOPSY CASE OF PRIMARY OSTEOMYELOSCLEROSIS IN A 6‐YEAR‐OLD GIRL
Author(s) -
Nagai Kazunori,
Sugiyama Yoshihiko,
Isono Yuya,
Yoshida Kosaku
Publication year - 1969
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1969.tb00694.x
Subject(s) - medicine , pathology , bone marrow , anisocytosis , autopsy , spleen , extramedullary hematopoiesis , lymph , anemia , haematopoiesis , genetics , stem cell , biology
A case of a 6‐year‐old girl with primary osteomyelosclerosis was reported. Clinically she had general fatigue, anemia and hepato‐splenomegaly. The peripheral blood showed anemia with leuco‐erythroblastic picture, anisocytosis and tear‐drop poikilocytosis of the red cells, and marked thrombocytosis. Diagnosis of osteomyelosclerosis was established by biopsies of the liver and bone marrow which showed dry tap aspiration. The patient died 1 year and 2 months after the onset of symptoms. Autospy findings revealed marked flbrillosis, proliferation of all 3 hematopoietic systems and moderate new formation of bone in the bone marrow. Extramedullary hematopoiesis associated with flbrillosis was found in the liver, spleen, lymph nodes, thymus, adrenal glands, lungs and kidneys. There were multiple thrombi in the inferior vena cava, left renal vein, pulmonary vessels, both suprarenal arteries with infarcts in the adrenal glands. It is considered that osteomyelosclerosis is not a reactive or reparative process in nature but a systemic autonomous progressive blood disease. A possible relationship between abnormality of the thymus and primary osteomyelosclerosis was suggested.