Jednoczesne wystąpienie transformacji przewlekłej białaczki limfocytowej do chłoniaka Hodgkina i raka płuca – opis przypadku i przegląd literatury
Concomitant occurrence of Hodgkin variant of Richter syndrome and a lung cancer in a patient with CLL – a case report and review of the literature
The course of B-cell chronic lymphocytic leukaemia (CLL) is variable: indolent in about one third of patients never requiring treatment. Approximately one-third represents an initially indolent disorder that progresses and requires therapy within 5 years after diagnosis. In remaining patients CLL is more aggressive disease requiring treatment but disease still maintains its characteristic phenotype (1).
However a small proportion of cases even with an indolent disease may transform to high grade aggressive lymphomas (2) first described in 1928 by Maurice N. Richter (3) who reported a case of a 46-year-old man with diffuse lymphadenopathy, massive organomegaly and a rapidly fatal clinical course as seen in untreated diffuse large B-cell lymphoma (DLBCL). Patients with Richter transformation (RT) typically present with constitutional symptoms (lost of weight, fever), rapidly enlarging lymph nodes, spleen or liver and increased lactate dehydrogenase (LDH) activity with histological features of DLBCL that are formally required to make a diagnosis of RT (4). The current estimated rate of Richter transformation in CLL is approximately 2-8% (4, 5). Although the most common type of histological transformation of CLL is to a high-grade B-non-Hodgkin lymphoma (NHL), other types of lymphomas have also been described, including Hodgkin lymphoma (6), small noncleaved cell lymphoma (7), lymphoblastic lymphoma (8), hairy cell leukaemia (9), and high-grade T-cell NHL (10, 11). Transformation of CLL/SLL to Hodgkin lymphoma, called ”Hodgkin variant of Richter transformation (HLvariantRT) occurs very rarely (0.5% of patients with CLL/SLL) constituting 15% of RT. (6, 12-14) Although rare, it is considered one of the commonest second malignancies in patients with a known CLL. Increased risk of developing Hodgkin lymphoma among CLL patients when compared with the general population, with an observed-to-expected ratio of 7.69 was reported by Travis et al. (15).
Patients with CLL have also an increased risk (20%) of second malignancy compared to general population (15, 16). Significantly increased risks were observed for Kaposi sarcoma, malignant melanoma and cancers of the larynx and the lung (16). Presentation of second malignancy in patients with CLL may mimic RT symptoms causing delay in correct diagnosis, thus decreasing a chance of early intervention. Here we describe diagnostic and therapeutic difficulties in a 59-year old patient with CLL, who almost simultaneously developed non small cell lung cancer and Hodgkin variant of Richter syndrome two years after the diagnosis of CLL.
In February 2007 on physical examination, the patient was found an oedema of the left lower extremity, followed few days later by diarrhoea and fever of up to 39°C with shivers. The patient was admitted to the department for internal medicine. His WBC was stable (14.82 x 109/l, lymphocytosis 6.84 x 109/l) and haemoglobin and platelets were within normal range. Chest X-ray revealed a round lesion of approx. 15 mm in the right apex of the lung and a polycyclic right hilum of the lung. Radiographic evaluation including comparison with prior films suggested that the lesion in the apex of the right lung might be related to a putative past tuberculosis, whereas a widening of pulmonary hilum to the primary disease. Doppler test of deep veins in the left lower extremity indicated thrombosis in the sagittal vein, 1/2 of the distal femoral vein and popliteal veins. Following an antithrombotic therapy and administration of antibiotics, oedema of the left lower extremity disappeared with concomitant improvement of the patient’s general condition. Chlorambucil and prednisone were re-introduced. The patient received two subsequent cycles and his general condition remained stable.
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