[Mast cell leukemia--case report]. / Mastocitna leukemija--prikaz bolesnice.

UNLABELLED: Mast cell leukemia is extremely rare, more often arises de novo and in 15% of cases developes from preexistence mast cell diseases. It is a high malignant leukemia with bad prognosis and a short survival. A 72-year old female was admitted to hospital with clinically suspected plasmocytoma. She had eccematoid changes on her trunk and underarms. Radiogramm showed osteolytic lesions of the right thigh and pelvis. FNA of the bone marrow revealed hypercellular smear with 80% of mast cells and immature mast cells which were cytochemically positive to toluidin (Figure 1. and 2.), chloracetate, alcian blue, acid fosphatase and Sudan black and negative to periodic acid Schiff (PAS) and peroxidase (POX). The peripheral blood smear showed single mast cells which were positive to toluidin. The bone marrow biopsy confirmed mast cell leukemia. The chariogramm of the bone marrow showed numerical and structural chromosomal changes. Two month after the diagnosis the patient suffered from right thigh fracture and was treated with local radiotherapy, antihistaminic and analgetic therapy. The patient died within 6 month after the diagnosis. CONCLUSION: Mast cell leukemia is very rare and high grade leukemia with short survival time. Cytological diagnosis is possible, when there is more then 20% of atypical mast cells in the bone marrow aspirate. The diagnosis of aleukemic variant of mast cell leukemia could be stated if there is less then 10% of mast cells in the peripheral blood.

Hodgkin's lymphoma variant of Richter's syndrome.

Chronic lymphocytic leukemia/small lymphocitic lymphoma (CLL/SLL) is low-grade malignant lymphoprolipheration, that has tendency to convert to a higher-grade neoplasm over time. More common is the development of a diffuse large cell lymphoma or transformation into prolymphocytic cell population. In rare cases, 0.1-0.5% of patients develop multiple myeloma or Hodgkin's disease. We present 65-year-old female with Hodgkin's variant of Richter's syndrome. On the basis of clinical simptoms, cytological, hystological and immunohistological finding in April 2008 CLL/SLL were diagnosed. The patient was treated with 8 courses of R-CHOP. After 10 months, FNA of the one of the enlarged lymph node on the neck was performed. The diagnosis was Hodgkin's disease. Immuno-hystological studies of the lymph node was consistent with type I Hodgkin's type of Richter's syndrome. Patient was treated with 3 courses of ABVD and radiotherapy.

First report of connexin 43-positive gastrointestinal stromal tumor (GIST).

Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms most frequently found in the stomach and presenting without symptoms or with unspecific ones such as hemorrhage and abdominal pain. The malignant potential of GIST is variable and there are several prognostic indexes for treatment and follow up. The superior diagnostic method is endoscopic ultrasound combined with fine needle aspiration biopsy (EUS-FNA) immunocytochemistry. Surgery is the preferable treatment option, while recurrent or advanced disease is best managed with thyrosine kinase inhibitors. We report a case of a 20-year-old man that presented with gastrointestinal bleeding and anemia. Upper gastrointestinal endoscopy detected a submucosal lesion and computerized tomography revealed a node near the liver. EUS-FNA immunocytochemistry found CD-117 positive cells suggestive of GIST and the patient was operated on. The diagnosis was confirmed by histopathology with immunostaining. Prognostic assessment was done according to tumor size and Ki-67 index with mitosis count on microscopy. Many studies have shown that tumors demonstrate an abnormal number, structure and occurrence of connexin proteins with altered connexin-mediated intercellular communication. Since a great deal of gastroenterological tumors express connexin 43, our aim was to find out whether it was present in GIST. Immunohistochemically, the tumor was positive for connexin 43, which was not previously described as typical. The early postoperative course was uneventful, free from complications. At three-year postoperative follow-up, the patient was subjectively well and without clinical signs of disease recurrence.