Comprehensive mastocytosis data analysis from a single center.

Mastocytosis is a very rare disorder and is divided into three prognostically distinct variants by World Health Organization: Cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mast cell sarcoma or localized mast cell (MC) tumors. The wide range of complaints may cause patients to consult various clinics, with resulting mis- or underdiagnosis. Therefore, cooperation between different subspecialties is of paramount importance. In this article, we have compiled 104 adult mastocytosis cases diagnosed and followed in our Hematology and other clinics. 86 (82.7%) of 104 patients had systemic mastocytosis. Osteoporosis, disease-related complications, and secondary malignancies are important topics in this group. We know that indolent form has great survival. But smoldering or aggressive mastocytosis has a poor prognosis. CM and indolent SM have a significantly better prognosis compared to aggressive SM (p < 0.001). We found that the presence of more than 25% of mast cells in the bone marrow, the presence of concomitant marrow dysplasia, and the presence of disease-related complications affect survival (p < 0.001). In addition to the WHO classification, the IPSM scoring system is indicative of the prognosis in this rare disease.

Primary B-Cell Mucosa-Associated Lymphoid Tissue Lymphoma of the Hard Palate and Parotid Gland: Report of One Case and Review of the Literature.

A 61-year-old woman was admitted to our hospital with an ulcerated palate mass and swelling of the right parotid gland. Incisional biopsy from the hard palate revealed an extranodal marginal zone B-cell lymphoma, also called mucosa-associated lymphoid tissue (MALT) lymphoma. Final diagnosis was MALT lymphoma of the parotid gland with concomitant involvement of an extremely seldom site of involvement: the hard palate. To our knowledge, this report illustrates the first case of MALT lymphoma of the hard palate and parotid gland without an underlying autoimmune disease. Rituximab-based combination regimen (R-CHOP) provided complete remission with total regression of mass lesions at the hard palate and parotid gland. At 44-month follow-up, there is no disease relapse. We adressed the manifestations and management of MALT lymphoma patients with involvement of salivary gland and oral cavity.

MALT lymphoma of the left biceps muscle: a rare case with an interesting presentation.

BACKGROUND: Extranodal marginal zone lymphoma (MZL), also called mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 7-8 % of non-Hodgkin lymphomas (NHLs) and most commonly involves the stomach. However, muscle involvement is very rare. CASE DESCRIPTION: A 57-year-old woman was referred to our orthopaedics and traumatology clinic with a painful lump in the left arm. Physical examination revealed a red-colored mass on the left arm and an enlarged lymph node measuring almost 5 cm in the left axillary region and 3 cm in the right axillary region. Tru-cut biopsy of the mass in the left arm was consistent with MZL. The diagnosis was MALT lymphoma infiltrating the skeletal muscle (stage IIEA). R-CHOP was started. Two additional infusions of rituximab were administered after the sixth cycle of R-CHOP. Then, the patient received radiotherapy to the left arm at a dose of 30 Gy. After 1 year of follow-up, the patient had no evidence of disease. DISCUSSION AND EVALUATION: MALT lymphoma arises in a number of epithelial tissues. The clinical presentation of MALT lymphoma varies depending upon the tissue involved. To our knowledge, rare cases of MALT lymphoma of the skeletal muscle have been reported. Although the available literature suggests that primary skeletal muscle NHL with advanced stage is associated with poor prognosis, the case presented here suggests that rituximab based combination therapy followed by radiotherapy can be an effective treatment for primary skeletal MALT lymphoma. CONCLUSION: There is limited data regarding the prognosis and treatment of MALT lymphoma of the skeletal muscle. This case implies that rituximab based combination therapy followed by radiotherapy should be considered for the treatment of primary skeletal MALT lymphoma.

Acute Severe Hepatitis and Hemophagocytosis in Adult Onset Still's Disease.

We report a 44-year-old male Turkish patient with adult onset Still's disease (AOSD) complicated by acute severe hepatitis and hemophagocytosis. Initial investigations for fever and rapidly progressive elevation of liver function tests were not diagnostic. Routine evaluations of liver and bone marrow biopsies missed the fundamental pathology. Extremely elevated ferritin levels led to a more detailed search, and immunohistochemical staining with CD68 for macrophages revealed extensive hemophagocytosis in both the first and second bone marrow biopsies, as well as in the liver biopsy. Treatment with steroid and cyclosporine A induced complete remission.

Hairy Cell Leukemia Presenting with Isolated Skeletal Involvement Successfully Treated by Radiation Therapy and Cladribine: A Case Report and Review of the Literature.

We describe an unusual case of hairy cell leukemia (HCL) in a 55-year-old male presenting with isolated skeletal disease as the initial manifestation without abnormal peripheral blood counts, bone marrow involvement, or splenomegaly. To the best of our knowledge, there have been only two previous reports of a similar case. The patient presented with pain in the right femur. Anteroposterior radiographs of both femurs revealed mixed lytic-sclerotic lesions. PET scan showed multiple metastatic lesions on axial skeleton, pelvis, and both femurs. Histopathological examination of the bone biopsy revealed an infiltrate of HCL. Localized radiation therapy to both proximal femurs and subsequently 4 weeks later, a 7-day course of 0.1 mg/kg/day cladribine provided complete remission with relief of symptoms and resolution of bone lesions. We addressed the manifestations and management of HCL patients with skeletal involvement.