C1 esterase inhibitor in pregnancy

Abstract

This patient had multiple diagnoses, including lymphadenopathic mastocytosis with involvement of the spleen, liver, abdominal lymph nodes, skeleton, bone marrow, skin, and gastrointestinal tract. He was also diagnosed with acute nonlymphocytic leukemia predated by the discovery of a biclonal gammopathy. 3 Oligoclonal immunoglobulins have been observed in systemic mastocytosis, but this is the first patient with systemic mastocytosis to our knowledge with a biclonal gammopathy. 20l igoclonal imrnunoglobulins were found to be associated with those patients who had a secondary malignancy as well as a more malignant form of mastocytosis. 2 Biclonal gammopathy most commonly occurs as an IgG/IgA combination, and a significant portion (16%) of these patients are diagnosed with lymphoproliferative disorders such as lymphoma and lymphocytic leukemia, s As a group, patients with systemic mastocytosis have an abnormally increased incidence of hematologic disorders and malignancies including dysmyelopoietic syndromes, myeloproliferative disorders, acute leukemia, and malignant lymphoma. Patients with systemic mastocytosis and coincident hematologic disorders have a significantly decreased survival time compared with the patients with systemic mastocytosis alone. 6 We have presented this brief communication to provide a follow up of our Allergy Grand Rounds presentation on a patient with lymphadenopathic mastocytosis and biclonal gammopathy. 3 Mastocytosis has been suggested to be due to an innate oncogenic aberration in a hematopoietic stem cell resulting in abnormal mast cell proliferation. 2 The development of leukemia could correspond to an increased proliferation of cellular forms that possess this aberration. 2 Perhaps, T-cell derived mast cell interleukin growth factors (IL-3, IL-4, IL-9, IL-10) are partly involved in regulating the expression of neoplasia in a subset of patients with systemic mastocytosis. 3 Finally, this case integrates biclonal gammopathy with systemic mastocytosis and the likelihood of developing malignancy.