MAST CELL LEUKEMIA - CASE REPORT

Mastocitna leukemija je iznimno rijetka, češće nastaje de novo, a oko 15% ih se razvija iz ostalih oblika mastocitoza. Definira se kao visoko-maligna leukemija s lošom prognozom i kratkim preživljenjem. Prikazujemo 72-godišnju bolesnicu, koja je zaprimljena u bolnicu zbog kliničke sumnje na plazmocitom. Pri pregledu nađene su ekcematozne promjene na koži trupa i podlaktica, te slabija pokretljivost. Radiološke slike kostiju pokazale su osteolitičke lezije zdjelice i desne natkoljenice. Punkcijom koštane srži dobivena je hipercelularna koštana srž u kojoj 80% stanica čine mastociti i atipične nezrele mastocitne stanice koje su pozitivne na toluidin, kloracetat, alcian-blu, kiselu fosfatazu i Sudan black, a PAS (Periodic acid schiff) i POX (peroksidaza) su negativni. U razmazu periferne krvi također su nađeni pojedinačni mastociti. Na osnovi morfologije stanica i citokemijskih reakcija postavljena je citološka dijagnoza mastocitne leukemije. Učinjena je i biopsija kosti koja potvrđuje citološku dijagnozu. Bolesnica je liječena antihistaminicima i analgeticima. Dva mjeseca nakon postavljene dijagnoze došlo je do prijeloma prethodno bolne desne natkoljenice nakon čega je provedena lokalna radioterapija. Bolesnica je umrla unutar 6 mjeseci od postavljanja dijagnoze. Zaključujemo da, iako iznimno rijetka, mastocitna leukemija se citološki može dijagnosticirati kada se u koštanoj srži nađe više od 20% patoloških oblika mastocita uz njihovu pojavu u perifernoj krvi.Mast cell leukemia is extremely rare, more offen 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 conwert to a higher-grade neoplasam 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 month, 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 radiotherap

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 conwert to a higher-grade neoplasam 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 month, 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 radiotherap

Prvi prikaz slučaja probavnog stromalnog tumora (GIST) pozitivnog na koneksin 43

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.Gastrointestinalni stromalni tumor (GIST) spada u novotvorine mezenhimnog podrijetla, a najčešće se nalazi u želucu. Često bude bez simptoma ili se prezentira paletom nespecifičnih simptoma poput gastrointestinalnog krvarenja ili boli u abdomenu. Maligni potencijal GIST-a je raznolik te postoji nekoliko prognostičkih indeksa za dijagnostiku i praćenje. Najznačajniju dijagnostičku metodu čini endoskopski ultrazvuk kombiniran s citološkom biopsijom. S terapijske strane najpoželjniji izbor je kirurško liječenje, a za uznapredovale ili recidivne oblike bolesti liječenje je farmakološko inhibitorima tirozin-kinaze. Ovdje se prikazuje slučaj 20-godišnjeg mladića koji je došao na obradu zbog gastrointestinalnog krvarenja i anemije. Gastroskopijom se otkrila submukozna promjena koja se na kompjutoriziranoj tomografiji prikazala kao čvor uza stražnji rub jetre. Citopunkcijom uz pomoć endoskopskog ultrazvuka nađene su stanice pozitivne na CD-117 koje ukazuju na GIST, te je bolesnik operiran. Dijagnoza je potvrđena patohistološki uz imunohistološko bojanje. Prognostička patohistološka procjena je učinjena prema veličini tumora, Ki 67 mitotskom indeksu. Brojne studije su pokazale kvantitativne ili kvalitativne promjene koneksinskih proteina te promijenjene međustanične komunikacije posredovane tijesnim vezama. Kako je u velikom broju tumora probavne cijevi primijećena promjena ekspresije koneksina Cx43, naš je cilj bio ispitati zastupljenost u GIST-u. Naš slučaj bio je pozitivan na Cx43, što nije ranije opisano kao očekivano. Rani poslijeoperacijski tijek u bolesnika je bio bez komplikacija, a dosad se kroz ambulantno praćenje unatrag 3 godine osjeća dobro, kontrolni nalazi labolatorija su uredni, te se kliničkom obradom ne nalazi znakova recidiva bolesti