Rozlany chłoniak złośliwy typu B przebiegający z naciekiem skrzyżowania wzrokowego, zaburzeniami widzenia, niedoczynnością przysadki, hiperprolaktynemią i moczówką prostą. Opis przypadku i przegląd literatury

The case is reported of a 55-year-old man with diffuse malignant lymphoma type B associated with transient optic chiasm infiltration and visual disturbances but with persistent hypopituitarism, hyperprolactinaemia and diabetes insipidus. The patient was administered chemotherapy and radiotherapy. Repeated MR and CT scans showed optic chiasm infiltration, which disappeared in the course of the chemotherapy but then recurred, changed its appearance and finally disappeared again. In the meantime visual disturbances occurred and disappeared during the therapy. Hypopituitarism, diabetes insipidus and hyperprolactinaemia were diagnosed and replacement therapy was administered. Later on abdominal pain occurred, and a CT scan revealed bilateral kidney masses and enlarged retroperitoneal lymph nodes. These were diffuse malignant lymphoma with regional lymphonodulitis in histology. Finally, hydrothorax and hydroretroperitoneum were diagnosed. The patient died as a result of systemic complications of the disease. The length of survival time documented following the hypothalamochiasmatic infiltration and diagnosis of lymphoma makes the case an unusual one for patients with CNS lymphoma. Hormonal disturbances accompanying the suprasellar region infiltration are very important from the practical point of view.Przedstawiono opis przypadku 55-letniego mężczyzny chorującego na rozlanego chłoniaka złośliwego typu B z towarzyszącym przemijającym naciekiem skrzyżowania wzrokowego i zaburzeniami widzenia oraz przetrwałą niedoczynnością przysadki, hiperprolaktynemią i moczówką prostą. Chorego leczono chemio- i radioterapią. Powtarzane badania za pomocą jądrowego rezonansu magnetycznego (NMR, nuclear magnetic resonance) i tomografii komputerowej (TK) wykazywały naciek skrzyżowania nerwów wzrokowych, który ustępował w wyniku chemioterapii i znowu nawracał, zmieniał swój charakter i w końcu wycofał się. Równocześnie pojawiły się zaburzenia widzenia, które również ustąpiły w czasie leczenia. Stwierdzono wymagające substytucji niedoczynność przysadki i moczówkę prostą, a także hiperprolaktynemię. W późniejszym czasie dołączyły się objawy brzuszne i w badaniu TK stwierdzono obustronne zmiany w nerkach i powiększenie pozaotrzewnowych węzłów chłonnych. W badaniu histolopatologicznym potwierdzono rozpoznanie rozlanego chłoniaka złośliwego i miejscowe zapalenie węzłów chłonnych. Następnie wykazano obecność płynu w jamie opłucnej i przestrzeni pozaotrzewnowej, a pacjent zmarł z powodu powikłań choroby zasadniczej. W opisywanym przypadku udokumentowano długie przeżycie od czasu rozpoznania nacieku okolicy podwzgórza i skrzyżowania wzrokowego w przebiegu chłoniaka, co jest rzadkością w chłoniakach ośrodkowego układu nerwowego. Z praktycznego punktu widzenia ważne są zaburzenia hormonalne towarzyszące nacieczeniu okolicy nadsiodłowej

Application of the apparent diffusion coefficient in magnetic resonance imaging in an assessment of the early response to treatment in Hodgkin's and non-Hodgkin's lymphoma : pilot study

Purpose: Lymphoproliferative neoplasms are the largest and most frequently diagnosed entities in the group of haematological malignancies. The aim of the study was to assess whether apparent diffusion coefficient (ADC) measured on the first day of the second cycle of chemotherapy could be a predictor of prognosis and of the final treatment's outcome. Material and methods: The study included 27 patients with diagnosed Hodgkin's and non-Hodgkin's lymphoma, who had magnetic resonance (MR) performed with diffusion weighted imaging/apparent diffusion coefficient (DWI/ADC) before and on the first day of the second cycle of chemotherapy. Imaging was performed using a 1.5 T MR scanner. ADC was measured in lymphoma infiltration in the area of the lowest signal in the ADC map and the highest signal on b 800 images in post-treatment study. After that, the corresponding area was determined in a pre-treatment study and an ADC value was measured. Results: The difference between ADC values in pre-treatment (ADC = 720 mm2/s) and post-treatment (ADC = 1059 mm2/s) studies was statistically significant (p 752 mm2/s before treatment manifested lower probability of progression than patients with ADC < 752 mm2/s. Conclusions: ADC measurement's before treatment and on the first day of the second cycle of chemotherapy can be used as a prognostic marker in lymphoma therapy. ADC values lower than 1080 mm2/s and an increase of the ratio after the treatment can be considered as a marker of disease progression

A risk of essential thrombocythemia in carriers of constitutional CHEK2 gene mutations

Germline mutations of the CHEK2 gene have been reported in some myeloid and lymphoid malignancies, but their impact on development of essential thrombocythemia has not been studied. In 16 out of 106 (15.1%) consecutive patients, newly diagnosed with essential thrombocythemia, we found one of four analyzed CHEK2 mutations: I157T, 1100delC, IVS2+1G>A or del5395. They were associated with the increased risk of disease (OR=3.8; P=0.002). The median age at ET diagnosis among CHEK2+/JAK2V617F+ patients was seven years lower than that among CHEK2−/JAK2V617F+ (52 vs. 59 years; P=0.04), whereas there was no difference in the medians of hematologic parameters between these groups. The results obtained suggest that CHEK2 mutations could potentially contribute to the susceptibility to essential thrombocythemia. The germline inactivation of CHEK2, as it seems, has no direct impact on the development of disease, but it could cause disruption of cell cycle checkpoints and initiate or support the cancerogenic process of essential thrombocythemia at a younger age