Pure red cell aplasia associated with malignant thymoma, myasthenia gravis, polyclonal large granular lymphocytosis and clonal thymic T cell expansion.
A case with the triad of pure red cell aplasia (PRCA), myasthenia gravis, and malignant thymoma is reported. There was a clonal proliferation of T cells within the thymoma, as demonstrated by a T cell antigen receptor (TCR) delta chain gene rearrangement. However, despite a large granular lymphocytosis, clonality could not be shown in the peripheral blood either before or after thymectomy. There was no evidence of human T cell lymphotrophic virus type 7 (HTLV1) infection. It is postulated that the clonal thymic T cell population secreted cytokine(s), which stimulated the polyclonal proliferation of large granular lymphocytes, which in turn suppressed erythropoiesis. Thymectomy removed the stimulus to the large granular lymphocytes and hence there was a resurgence of erythropoiesis.
Handa
S I
SI
Department of Haematology, North Manchester General Hospital.
Schofield
K P
KP
Sivakumaran
M
M
Short
M
M
Pumphrey
R S
RS
D000368:Aged; D001345:Autoradiography; D002999:Clone Cells; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D008213:Lymphocyte Activation; D008218:Lymphocytosis / Q000150:complications*; D008297:Male; D009157:Myasthenia Gravis / Q000150:complications*; D012010:Red-Cell Aplasia, Pure / Q000150:complications*; D013601:T-Lymphocytes / Q000502:physiology*; D013934:Thymectomy; D013945:Thymoma / Q000150:complications*; D013953:Thymus Neoplasms / Q000150:complications*