Syndrome of neutrophil agranulocytosis, hypogammaglobulinemia, and thymoma

Abstract

The clinical, hematologic, and immunologic findings of a syndrome of agranulocytosis, hypogammaglobulinemia, and thymoma are described. Neutrophil agranulocytosis predisposing to severe infectious disease resulted from a deficiency of mature cells in bone marrow. Autologous and heterologous stem cell growth in vitro was inhibited by the patient's serum. Immunoglobulin deficiency was secondary to the absence of peripheral blood B lymphocytes, while T-cell subpopulations and cellular immunity were present. Surgical removal of a spindle cell thymoma had no effect on the agranulocytosis and B-cell deficiency. The hematologic findings did not respond to steroid therapy and cyclophosphamide. However, the agranulocytosis improved with repeated plasmapheresis and the patient achieved a clinical remission.

Complete remission of pure white cell aplasia associated with thymoma, autoimmune thyroiditis and type 1 diabetes

“…However, absence of myelopoiesis, as in our case, has been described in only four cases (10–13). Bone marrow showed a promyelocyte arrest in five other cases (2, 9, 13–15).…”

“…PWCA was first described in association with an inhibitory activity of immunoglobulins (Ig) G and M on granulopoiesis (1). Association with thymoma was reported later (2), as well as evidence of an inhibitory effect of circulating IgG on granulocyte‐monocyte forming units (2, 3). There are only four descriptions of primary PWCA (3–6).…”

Pure White Cell Aplasia an exceptional condition in the inmunological conundrum of thymomas: Responses to inmunosupresion and Literature Review.

“…It has been reported the case of a patient with thymoma and granulocytopenia in whom a decrease in anti-pANCA antibody titre and elevation of granulocytes in peripheral blood was observed after thymectomy 9 . However, in other cases neutropenia has not resolved after thymectomy and a second line of treatment is necessary 10,11 . The medical treatment of these patients is not established currently, due to low incidence of cases.…”

Prolonged remission of pure white cell aplasia (PWCA), in a patient with CLL, induced by rituximab and maintained by continuous oral cyclosporin

“…Despite the effectiveness of rituximab in pure red cell aplasia and immune thrombocytopenia (6, 7), our patient’s response was transient. The temporary response to IVIg and rituximab, alludes to the role of humoral immunity as the cause of PWCA despite the absence of anti‐neutrophil antibodies and inconclusive effect of patient’s serum on CFU‐GM (8).…”