T-Cell Large Granular Lymphocytic Leukemia - Case Reports.

T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Here we present a case of a 53-year-old male admitted to the hospital because of abdominal pain. Blood examination revealed mild mycrocitic anemia and multiplied lactate dehydrogenase level. Abdominal ultrasound showed splenomegaly of 16 cm, with no lymphadenopathy. Fine needle aspiration of bone marrow revealed hypocellular marrow with 50% of atypical lymphoid cells. There were 81% of atypical medium sized granular lymphocytes with irregularly shaped nuclei in peripheral blood, so the cytologic diagnosis was lymphoproliferative process. Bone marrow biopsy showed nodular and interstitial proliferation of small, partially atypical T lymphocytic cells positive for CD2, CD3, CD5, CD8, granzyme and TIA, and negative for hairy cell markers, CD10, MUM 1, bcl 1, CD4 and CD56. The finding was consistent with T-LGLL. Due to splenomegaly, the patient was treated with cyclosporine and gradually reduced dose of corticosteroids, leading to regression of splenomegaly and normalization of lactate dehydrogenase level.

FINE NEEDLE ASPIRATION CYTOLOGY OF ABDOMINAL ORGANS--TEN-YEAR SINGLE CENTER EXPERIENCE.

The aim of the study was to assess the role of fine needle aspiration cytology (FNAC) in the diagnosis of focal changes in solid abdominal organs. A total of 1084 aspirates from intra-abdominal organs including liver, spleen, pancreas and kidneys obtained by ultrasound (US) guidance during a 10-year period were included in the study. The smears were classified as benign, malignant or suspected of malignancy, and unsatisfactory for interpretation. The liver accounted for more than half of the US-guided FNA procedures, followed by the pancreas with 38%. Out of 1084 aspirations, 192 (17.7%) were inadequate for cytologic analysis. Over half of aspirated lesions in the pancreas were primary cancers, while one-third of pancreatic lesions were benign. In the majority of kidney lesions (83%), cytology found benign changes, mostly cysts. Spleen FNA was least likely; in most cases (59%) it showed lymphoid tissue hyperplasia; in four cases cytologic diagnosis was lymphoma and three lesions were suspected lymphoma. During the study, no major complications were observed on any US-guided FNAC procedure. In conclusion, intra-abdominal FNA is a reliable, sensitive and specific method with a high diagnostic accuracy for the diagnosis of malignant lesions. It can be utilized as a preoperative procedure for the management of all intra-abdominal lesions.

FINE-NEEDLE ASPIRATION CYTOLOGY OF HEAD AND NECK LYMPH NODES IN A TEN-YEAR PERIOD - SINGLE CENTER EXPERIENCE.

A wide spectrum of diseases including reactive processes, infections, lymphomas and metastatic tumors can cause enlargement of lymph nodes. The present study on 4062 patients with lymphadenopathy was conducted in the Department of Cytology, Sestre milosrdnice University Hospital Center, Zagreb, during a 10-year period. Of 4062 patients with lymphadenopathy, 1624 were males and 2438 were females, age range from several months to 85 years. Of 4062 fine needle aspiration procedures, 232/4062 (5.7%) samples were nondiagnostic. Lymph node aspirates were benign in 2640/3830 (69%) and malignant in 1190/3830 (31%) cases. Hematologic disease (Hodgkin lymphoma and non-Hodgkin lymphoma) was diagnosed in 482/1190 (40.5%) and metastases in 708/1190 (59.5%) cases. Of 482 patients with hematologic disease, 48/482 (10%) had Hodgkin lymphomas and 434/482 (90%) non-Hodgkin lymphomas. In the group of malignant lymphadenopathy, there were 212/708 (30%) metastatic squamous cell carcinomas, 177/708 (25%) metastatic adenocarcinomas, 149/708 (21%) metastases of other carcinomas, and 170/708 (24%) metastases of other malignant tumors. The present study confirmed the fine needle aspiration cytology of enlarged head and neck lymph nodes to be an excellent first-line method to investigate the nature of the lesions.

[PREOPERATIVE DIAGNOSTICS OF THYROID MEDULLARY CARCINOMA WITH EMPHASIS ON CYTOMORPHOLOGICAL FEATURES AND DIFFERENTIAL DIAGNOSIS OF PRIMARY AND SECONDARY THYROID TUMORS]. / PREOPERACIJSKA DIJAGNOSTIKA MEDULARNOG KARCINOMA STITNJACE S OSVRTOM NA CITOMORFOLOSKE ZNACAJKE I DIFERENCIJALNU DIJAGNOZU PRIMARNIH I SEKUNDARNIH TUMORA STITNJACE.

Medullary thyroid cancer is a rare neuroendocrine neoplasm that arises from the parafollicular C cells that produce calcitonin, a hormone essential for the regulation of calcium metabolism. It accounts for 4%-10% of all thyroid cancers. In most cases (75%-80%), it is sporadic, while in other cases it is part of the multiple endocrine neoplasia (MEN) syndrome. Most often, medullary thyroid carcinoma is presenting as a solitary nodule. At the time of diagnosis, about half of the patients have enlarged cervical lymph nodes, while a small number of patients have distant metastases in the liver, lungs, bones and brain. If the tumor is hormone active, the patient may have systemic symptoms such as diarrhea or flushing. Ultrasonically, medullary carcinoma usually appears as a hypoechogenic node with marked vascularity and uneven contours that can sometimes contain microcalcifications, and in most cases is located in the upper poles of the thyroid. The sample obtained by fine needle aspiration is usually cellular, tumor cells are disseminated or arranged in poorly cohesive groups. They are most often plasmacytoid, but sometimes can also be spindled. The nuclei are eccentric and chromatin shows features of neuroendocrine tumors. Cytoplasms of tumor cells are abundant, triangular or polygonal, amphophilic, finely granulated and unsharply limited. Background is clean and sometimes amyloid can be found. Depending on the cytologic picture, differential diagnostic problems can be well differentiated thyroid tumors, primarily follicular neoplasm, lymphomas, poorly differentiated insular carcinoma, metastatic small cell carcinoma, mesenchymal tumors or melanoma. In case of differential diagnostic difficulties, of great help is to determine calcitonin immunocytochemically or in aspirate or serum. Medullary carcinoma may show low progression and long-time survival, but can also be a rapidly progressive tumor where survival is measured in months. Good prognostic indicators are younger age, female gender, tumor size, tumor localization only in the thyroid gland, and absence of local or distant metastases. In conclusion, medullary thyroid carcinoma is a rare tumor in which we still have to think in diagnosing thyroid nodules, mainly due to adequate preoperative preparation in terms of exclusion of pheochromocytoma in MEN syndrome. Cytologic picture in some cases is typical; however, immunocytochemical evidence of calcitonin is of great help to confirm morphological diagnosis.

[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.

[Croatian Society for Clinical Cytology guidelines for thyroid cytology]. / Smjernice u citoloskoj dijagnostici stitnjace Hrvatskoga drustva za klinicku citologiju.

The main purpose of thyroid FNA (fine needle aspiration) is to separate malignant and possibly malignant nodules from benign thyroid lesions. Every patient with thyroid nodule is a candidate for FNA. Before a decision to perform an FNA, a complete history, a physical examination directed to the thyroid and cervical lymph nodes, a serum thyrotropin level, and thyroid ultrasound should be obtained. Thyroid lesion with a maximum diameter greater than 1.5 cm or nodule of any size with sonographically suspicious features is an indication for FNA. Ultrasound-guided FNA of the thyroid is recommended. The requisition form that accompanies FNA should contain the identifying data, location and size of the nodule, and relevant laboratory and clinical data. FNA diagnosis of thyroid disease is a clinicocytologic diagnosis, and correlation with clinical findings is mandatory for success. Thyroid FNA classification scheme consists of a four diagnostic categories according to the risk of malignancy: benign lesions, indeterminate lesions according to malignancy, malignant tumors, and non-diagnostic. Ancillary studies (immunocytochemistry, RT-PCR, flow cytometry) are usually helpful in borderline cases.

Aplastic crisis induced by human parvovirus B19 as an initial presentation of hereditary spherocytosis.

The association between aplastic crisis and human parvovirus (HPV) B19 infection has been described in patients with hereditary spherocytosis (HS). Most cases of aplastic crisis in patients with HS induced by HPV B19 have been reported in children and adolescents. In this paper, we describe an aplastic crisis induced by HPV B19 in the 34 year old female as an initial presentation of HS. Although other viral illnesses cause some decompensation in HS, the anemia is rarely as profound as seen in acute HPV B19 infections.

Simultaneous occurrence of chronic lymphocytic and chronic myeloid leukemia.

The coexistence of chronic lymphocytic (CLL) and chronic myeloid leukemia (CML) in the same patient has only been reported occasionally. Most of these cases represent the patients who developed CLL during the course of CML. Reviewing the literature, only a few cases of simultaneous occurrence of CLL and CML were found. Here we represent a previously healthy 50-year old man in whom the diagnosis of CLL and CML was established by FNAB of the bone marrow. The diagnosis was then confirmed by histopathology, immunophenotypization of the peripheral blood and by a cytogenetic study of the bone marrow. Four years after the diagnosis the patient is well, with leucocytosis of 40 x 10(9)/L, and lymphocytosis of 93%.

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.

Cytological detection of lymphoma in Douglas aspirate.

Except in primary effusion lymphoma (PEL), serous effusions with lymphomatous cells in non-Hodgkin lymphoma (NHL) are not frequently seen as first manifestation of disease. In NHL lymphoplasmacytic lymphoma (LPL) the spleen, lymph nodes, and bone marrow are frequently sites of disease and this type of NHL is usually associated with a serum paraprotein of IgM type accompanied by the clinical syndrome of Waldenström macroglobulinemia. Our patient with NHL LPL type presented in this report had less frequently seen involvement of gastrointestinal tract and clinically was first manifested as effusion in Douglas space. Cytological evaluation as well immunoanalyses of effusion in such cases is essential, and various ancillary studies, because of differential diagnostic problems of lymphomas in serous effusions, first include reactive lymphocytoses and small round-cell tumors (SRCT). In our patient, cytology of effusion revealed cytomorphologically atypical lymphomatous cells with plasmocytoid differentiation. Immunocytochemical and flow cytometry analysis confirmed lymphoid cell differentiation and pathohistological diagnosis of CD20(+) LPL was set after pathohistological analysis of resected ileum.