Thymomas are often associated with autoimmune disorders, We report on a 45-
year-old female patient with thymoma and hypogammaglobulinemia (Good's synd
rome) who developed symptomatic macrocytic anemia (Hb 4.4 g/dl, MCV 112 fl)
and thrombocytosis (Plt 442 G/I). Besides hypogammaglobulinemia (IgG 589 m
g/dl), an inverted ratio of CD4(+)/CD8(+) cells was seen. The bone marrow b
iopsy showed a slightly hypercellular bone marrow with normal granulopoiesi
s, normal megakaryopoiesis and a mild dyserythropoiesis without any ring-si
deroblasts. The in-vitro stem cell culture from the bone marrow revealed an
atypical growth of macroclusters, reduced BFU-E and CFU-GEMM colony growth
, whereas the CFU-GM colony growth was within the normal range. The chromos
omal analysis showed a normal karyotype. The plasma vitamin B-12 and folate
levels were within normal ranges, and we could not detect any autoantibodi
es. These findings excluded the differential diagnoses pure red cell aplasi
a (PRCA) and pernicious anemia. After resection of the thymoma of mixed cel
l type, the macrocytic anemia and thrombocytosis disappeared. The clinical
course was complicated by a cerebral palsy and a life-threatening fungal se
pticemia after surgery. In the third year after thymectomy, hyporegenerativ
e macrocytic anemia and thrombocytosis reappeared and an immunosuppressive
treatment with prednisolone (1 mg/kg BW) was started. After initiation of t
he prednisolone therapy, reticulocyte counts increased and macrocytic anemi
a as well as thrombocytosis disappeared. The normalization of these laborat
ory parameters during glucocorticoid therapy suggests that in rare cases th
e constellation of macrocytic anemia, thrombocytosis and hypogammaglobuline
mia may be due to an underlying immunologic mechanism, (C) 2000 Wiley-Liss,
Inc.