Introduction: GD is the commonest lysosomal storage disease worldwide. The majority of the patients have Type1 GD which is the non-neuronopathic form of disease. There are data of increased risk of cancer in GD patients, such as: multiple myeloma and other haematological malignancies, hepatocellular carcinoma and renal carcinoma. Factors of cancerogenesis in GD are accumulation of bioactive lipids, alternatively activated macrophages, immune dysregulation, genetic modifiers underlying the GD, splenectomy and enzyme replacement therapy. Extra-osseous soft tissue masses are described in GD patients, like localised deposition od Gaucher macrophages (Gaucheroma). To the best of our knowledge, no other case of extra-osseous soft tissue sarcoma in association with GD has been described in literature. To present very rare case of high grade leiomiosarcoma in association with Gaucher disease (GD). Case report: The case concerns 81 years old female with leucopenia and thrombocytopenia since year 2000. In 2014 she was diagnosed with undifferentiated pleomorphic sarcoma with prominent inflammation on her thigh, which was not completely surgically removed. She was diagnosed with leucopenia, thrombocytopenia and splenomegaly in 2014 on control examination. Bone marrow biopsy was performed and histologically and immunohistochemically was diagnosed GD. The diagnosis was confirmed by enzyme activity test. In 2018 revision of pathohistological finding of thigh tumour was performed. High grade leiomiosarcoma was diagnosed. She is alive and refuses any treatment. Conclusion: GD is rarely diagnosed in older age. All soft tissue masses in GD should be carefully examined because of increased risk of cancer in GD patients.

Aim: Evaluation of the usefulness of flow cytometry (FCM) serous effusion analysis in a diagnosis of pediatric Non-Hodgkin lymphomas (NHL). Introduction: Serous effusions are often the first, life-threatening manifestation of pediatric NHL. FCM immunophenotyping of effusions with cytological analysis could help in diagnosis of NHL, and thus enable fast initiating of cytoreductive therapy. Material and Methods: FCM analysis of serous effusions obtained from 17 children and adolescents hospitalized in Mother and Child Healthcare Institute of Serbia under clinical suspicion of NHL using the standardized panel of monoclonal antibodies: CD19, iCD79a, CD20, CD10, iIgM, kappa/lambda, iCD3, sCD3, CD7, CD2, CD5, CD4, CD8, CD1a. Cytological examination was performed on May-Grunwald-Giemsa stained slides. The results were correlated with histopathological findings of available tumor biopsies. Results: Precursor T-cell (T-III/T-IV) phenotype was confirmed in 5 samples. In 7/9 samples with mature B (B-IV) phenotype, FAB L3 cytomorphology indicated Burkitt lymphoma (BL), and in 2/8 suggested diffuse large B-cell lymphoma (DLBCL). Tumor biopsy was available in 7/14 patients and in all cases preliminary diagnosis was confirmed. In 3 patients with no malignant cells in effusions, FCM and cytomorphologicaly only reactive changes were observed, and diagnosis had to be made by tumor biopsy (BL 2 patients, DLBCL 1 patient). Out of 7 patients diagnosed only by FCM and cytological analysis, 6 achieved a remission of the illness. Conclusion: FCM detects NHL cells in malignant serous effusions fast and accurate. In combination with cytological analysis, FCM is sufficient for diagnosis in most cases, allowing rapid initiation of therapy.

Aim: We present a rare case of simultaneous occurrence Concurrent occurrence of acute myeloid leukemia (AML) and monoclonal plasmacitosis (MP): Introduction: The simultaneous occurrence of acute myeloid leukemia (AML) and monoclonal plasmacytosis (MP) in bone marrow (BM) biopsy in patient without previous exposure to chemotherapy is very rare. Case report: We reported the case of a 45-year-old woman who presented with fatigue, fever, maculopapular rush, weight loss and bone pain. She was admitted to the Haematology Department of University CHC Bezanijska Kosa in November 2015. The complete blood count showed: white blood cell count 33x109/l (neutrophils 31%, myeloblasts 12%, monoblasts, promonocytes and monocytes 53%), hemoglobin 77gr/l, platelet count 34x109/l and 5% blastic cells in the peripheral smear. Serum immunoelectrophoresis showed increased monoclonal IgA (IgA 9,98, IgM 2,29, IgG 10,1), kappa/lambda 0,84, Beta2microglobulin 3,16. Biochemistry showed elevated creatinin level 93umol/l, uric acid 412 umol/l, high LDH 876U/l and sedimentation rate (50mm/h). Urine electrophoresis showed monoclonal heavy and light chains lambda type. Liquor immunophenotyping showed neuroleukemia. Skeletal survey showed no lytic lesions. The BM aspiration revealed around 10% plasma cells and BM biopsy showed infiltration by 20% monoclonal plasma cells (kappa-/lambda ) with 30% infiltration by cells with monocytes differentiation (without excess of blasts). Biopsy of skin lesion 17 MATERIA MEDICA • Vol. 34 • Issue 1, suplement 1 • april 2018. revealed myeloid sarcoma. Cytogenetic analysis detected normal karyiotype with FLT3 and NPM mutation. The patient was diagnosed as AML-M5, administered with induction therapy (DA Cytosar) and consolidation therapy (HiDAC) and she was successfully treated with allogenenic stem cell transplantation. She achieved and maintained complete remission. Conclusion: Monoclonal plasmacytosis of BM should be carefully examined due to possible association with other haematological malignancies

Aim and introduction: Pediatric nodal marginal zone lymphoma (NMZL) is a rare, but distinct subtype of NMZL with characteristic clinical presentation, pathohistological and molecular features, therapy and prognosis. Results: We report the case of a 15-year-old boy with no remarkable past history, presented with painless enlargement of left submandibular lymph node (LN) for three months. He was admitted to the University Children’s Hospital in Belgrade in May 2016. The cervical ultrasound demonstrated moderate left submandibular lymphadenopathy, but also mild enlargement of two right submandibular LNs (17x7mm, 14x7mm). Physical examination, chest radiography and abdominal ultrasound revealed no hepatosplenomegaly and lymphadenopathy elsewhere. The result of blood count test was normal. Biochemistry showed elevated uric acid 499 umol/l, AST 45U/l, ALT 98U/l, and sedimentation rate (65mm/h). Urea, creatinine, alkaline phosphatase, LDH and CRP were normal. The patient underwent left submandibular LN excisional biopsy. The size of the LN was 47x37x20mm. The histopathological examination revealed partial architectural effacement: follicular hyperplasia and nodular B-cell infiltration with features of progressive transformation of germinal centers (PTGC) in the form of fragmentation of follicles. A CD20 immunostain shows an abnormal expansion of the marginal zone with infiltration of interfollicular space. These B-cells were negative for CD3, CD5, CD23, EBV-LMP1, bcl-6, CD10, EMA, CD30, CD15, MUM-1, and positive for bcl-2 and IgD. A CD21/ CD23/ fascin immunostain showed an expanded and disrupted follicular dendritic cell meshwork. Ki-67 highlighted residual follicular polarisation and a low proliferation rate in the interfollicular areas. Based on these pathohistological findings it was concluded that LN likely represent reactive follicular hyperplasia with atypical marginal zone hyperplasia or possible PNMZL, with APSTRAKTI 95 MATERIA MEDICA • Vol. 34 • Issue 1, suplement 1 • april 2018. recommendation of polymerase chain reaction (PCR) analysis of clonality. Additional IGH PCR analysis demonstrated biclonal heavy chain gene rearrangement. These findings were consistent with PNMZL. After consultation with members of International BFM study group for non-Hodgkin lymphomas, followup was recommended without any treatment. The patient has remained disease free for 22 months since diagnosis. Conclusion: We presented a rare case of PNMZL with morphological features of PTGC, but immunohistochemistry and additional PCR clonality analysis were crucial for final diagnosis. This case represents a diagnostic and therapeutic challenge because of their rarity in the pediatric population.