Reza Setoodeh

Nonnecrobiotic necrobiotic xanthogranuloma as an initial manifestation of paraproteinemia and small lymphocytic lymphoma in a patient with Sjögren syndrome.

We report a unique case of periocular nonnecrobiotic necrobiotic xanthogranuloma in a 52-year-old white woman with Sjögren syndrome who was subsequently found to have an immunoglobulin G paraproteinemia and coexisting small lymphocytic lymphoma. Therapy with fludarabine, cytoxan, and rituximab (FCR) resulted in a dramatic resolution of her sicca symptoms and periocular xanthogranulomas. This case further illustrates the association of hematolymphoid disorders with cutaneous xanthogranulomatous disease and the importance of additional appropriate laboratory and radiologic investigation for the accurate diagnosis of an underlying malignancy.

Secondary T-lymphoblastic leukemia in a patient with hairy cell leukemia following cladribine therapy: report of an extremely rare case and review of the literature

Hairy cell leukemia, a rare B-cell lymphoproliferative neoplasm, accounts for about 2% of all leukemias. Bone marrow and spleen are typical sites of involvement. Patients most commonly present with splenomegaly and pancytopenia and a few circulating small- to medium-sized lymphoid cells with circumferential hairy projections (hairy cells). For years the common approach to treatment of hairy cell leukemia was splenectomy and α -interferon; complete remission was not easily reachable, and median survival was only 4 years. In the 1980s, with the introduction of the purine analogs pentostatin (2-deoxycoformycin) and cladribine (2-chlorodeoxyadenosine), the treatment strategy dramatically changed, resulting in improved prognosis for those with the disease. With these treatments, follow-up studies on patients with hairy cell leukemia showed a higher overall complete remission rate, ranging from 76 to 98%, and a prolonged median disease-free survival of 11 – 16 years [1]. However, an adverse outcome encountered in hairy cell leukemia aff ecting the overall survival is the subsequent development

Concurrent splenic diffuse red pulp small B-cell lymphoma and benign clonal proliferation of T-cell large granular lymphocytes.

Splenic diffuse red pulp small B-cell lymphoma (SDRPSBCL) is newly recognized primary B-cell lymphoma of the spleen [1]. t shows overlapping clinical features with splenic marginal zone ymphoma (SMZL), including massive splenomegaly, leukemic nfiltrate in the spleen, bone marrow and peripheral blood, and nfrequent B-symptoms. The diagnosis of SDRPSBCL is mainly based n histological examination of the spleen. In contrast to SMZL, DRPSBCL is characterized by diffuse infiltrate of the red pulp with onomorphic small to medium-sized B-cells [2]. The neoplastic B-cells show a nonspecific phenotype. Patients with SDRPSBCL typically have a relatively favorable clinical course. T-cell large granulocytic lymphocytic leukemia (T-LGL) is a chronic T-cell lymphoproliferative disorder usually with an indolent clinical course [1,3]. Patients with T-LGL typically present with neutropenia with or without anemia, moderate lymphocytosis and moderate splenomegaly. T-LGL is often diagnosed in patients with autoimmune disorders, especially rheumatoid arthritis. It is characterized by clonal proliferation of CD8+CD57+ cytotoxic T-cells. The diagnosis of T-LGL is based on the clinical features and demonstration of clonal expansion of abnormal CD8+ LGL cells by flow cytometry and molecular studies. The spleen and bone marrow are commonly infiltrated by T-LGL cells to a variable extent. T-LGL has occasionally been associated with B-cell lymphoproliferative