[Thymoma with pure red cell aplasia and hemophagocytic syndrome--one case report].
Erythropoietic hypoplasia occurring in the absence of abnormalities in the leukopoietic and thrombocytopoietic series is often defined as "pure red cell aplasia" (PRCA). This condition may appear as an acquired defect of either acute or chronic type, and a congenital form as well. The chronic form of acquired PRCA occurred mostly in adults. It has been reported that a demonstrable thymoma occurred in more than 50% of patients with PRCA. Recent studies suggested that it may contribute to several immune mechanisms. Here we report a case of thymoma with PRCA whose clinical presentations include severe anemia, shock with severe metabolic acidosis, high levels of several organ enzymes (SGOT, SGPT, LDH, CPK, Amylase) and acute renal shutdown with similar manifestations to septic shock. Our explanation to his condition is multi-organ tissue hypoxia caused by severe anemia. Hemophagocytic syndrome was found by the repeated bone marrow smear before death. The clinical course of this patient was so impressive as to be presented here for discussion.
Chuang
M W
MW
Section of Hematology-Oncology, Sha-Lu Tung General Hospital.
Lau
Y M
YM
Tung
J N
JN
Tung
J C
JC
Chen
P M
PM
D004920:Erythropoiesis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010587:Phagocytosis; D012010:Red-Cell Aplasia, Pure / Q000097:blood / Q000150:complications*; D013945:Thymoma / Q000097:blood / Q000150:complications*; D013953:Thymus Neoplasms / Q000097:blood / Q000150:complications*