4. Hrvatski kongres kliniËke citologije 4th Croatian Congress ... - Penta

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4. Hrvatski kongres kliniËke citologije / 1. Hrvatski simpozij analitiËke citologije / 2. Hrvatski simpozij citotehnologije 122 Klinička citologija - Posteri FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN A 6-WEEK-OLD MALE INFANT Jakovljević G1 , Kardum-Skelin I2 , Rogošić S1 , Čulić S3 , Stepan S1 , Bonevski A1 , Rimac M1 , Nakić M1 1Department of Hematology and Oncology, Pediatric Clinic, Children’s Hospital Zagreb, Zagreb, Croatia 2Laboratory for Cytology and Hematology, Department of Medicine, Merkur University Hospital, Zagreb, Croatia 3 Department of Pediatric Hematology and Oncology, Clinical Hospital Centre Split and Medical School University of Split, Split, Croatia Familial hemophagocytic lymphohistiocytosis (FLH) is an autosomal recessively inherited multisystem disease. This defect in cellular cytotoxicity is a life threatening condition characterized by fever, rash, splenomegaly, cytopenias and neurologic manifestations. PRF1, UNC13D and STX11 gene defects underlie in about 40-50% of primary cases. Chemoimmunotherapy followed by hematopoietic stem cell transplantation improved disease outcome. We report the case of a 6-week-old boy who presented with a fever, diffuse rash, disseminated intravascular coagulation, hypofibrinogenemia, hypertrigliceridemia, hepatosplenomegaly, leukocytosis with 90% of lymphocytes, granulocytopenia, anemia, trombocytopenia, hyperferritinemia and pathological findings in cerebrospinal fluid. The patient had decreased frequency of NK cells and low NK cell activity in peripheral blood. Bone marrow aspiration analysis showed degenerative changes of histocyte cells, with preserved cytophages (lymphophages and erythrophages) consistent with hematophagocytic syndrome. Given that the molecular diagnosis of the known mutations in genes PRF1 and UNC13D showed a mutation in UNC13D, the diagnosis of familial hemophagocytic lymphohistiocytosis subtype 3 was established. HLH-2004 chemotherapy protocol was performed and partial remission with residual central nervous system disease was achieved. Hematopoietic stem cell transplantation was successfully performed with an unrelated HLA-identical donor. Familiar HLH is generaly a progressive and fatal disease. Early diagnosis with molecular genetic analysis and chemoimmunotherapy followed by hematopoietic stem-cell transplantation is the best approach. gordanajakovljevic@yahoo.com
Clinical Cytology - Posters MYELOID SARCOMA INVOLVING THE BREAST Jelić Puškarić B1 , Ostojić-Kolonić S2 , Planinc-Peraica A2 , Obad-Kovačević D3 , Kardum-Skelin I1 , Jakšić B2 1Merkur University Hospital, Department of Medicine, Laboratory for Cytology and Hematology, Zagreb, Croatia 2 Merkur University Hospital, Department of Medicine, Zagreb, Croatia 3Merkur University Hospital, Department of Diagnostic and Interventional Radiology, Zagreb, Croatia Myeloid sarcoma is a tumor mass with extramedulary growth pattern, composed of myeloblasts or immature myeloid cells. The development of myeloid sarcoma may precede or concur with acute or chronic myeloid leukemia or other myeloproliferative diseases or myelodysplastic syndromes. Isolated myeloid sarcoma of the breast is very rare. A case is presented of a 25-year-old, previously healthy woman that presented to our department for a palpable node, 5x2 cm in size, in the upper medial quadrant of her left breast. Fine needle aspiration cytology (FNAC) FNAC produced a sample consisting of medium sized blasts of dispersed, gentle chromatin and basophile cytoplasm structure showing negative reaction to PAS, nonspecific esterase and myeloperoxidase; however, immunocytochemistry yielded positive reaction to myeloperoxidase and negative reaction to lymphocytic markers CD20, CD3 and CD10. Additional work-up revealed anemia (E 4.1x10/L), thrombocytopenia (53x109/L) and leukocytosis (10x109/L), along with atypical blasts detected in peripheral blood smear (76%). Bone marrow FNAC yielded a hypercellular sample of bone marrow with 97% of atypical blasts of the same cytochemical and immunocytochemical characteristics as the breast FNAC sample. On flow cytometry, the phenotype of the breast and bone marrow samples showed a very high percentage expression of the immature myeloid cell markers CD34, CD33 and HLA DR. Cytogenetic analysis of the breast and bone marrow samples indicated numerous alterations (81,XX,- X,-X,-7,-8,-8,-9,add(17p)x2,-18,-18[15]/46,xx[3]). Based on the morphology, cytochemical characteristics and immature cell immunophenotype, it was considered a case of acute myeloid leukemia without maturation, AML-M1. In spite of intensive chemotherapy, the patient died within a year of diagnosis. In cases of isolated breast myeloid sarcoma, the diagnosis can be missed if the possibility of myeloid sarcoma is not remembered on differential diagnosis of a breast neoplasm. Immunohistochemical studies are extremely helpful to recognize isolated myeloid sarcoma. biljana.jelic.puskaric@zg.t-com.hr 123 4 th Croatian Congress of Clinical Cytology / 1 st Croatian Symposium of Analytical Cytology / 2 nd Croatian Symposium of Cytotechnology
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Final programme and abstract book
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UTORAK, 13.10.2009. SRIJEDA, 14.10.
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4. Hrvatski kongres kliniËke citologije 4th Croatian Congress ... - Penta

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