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Mejstříková Ester, Sumerauer David, Froňková Eva, Mužíková Kateřina, Seeman Tomáš, Zemanová Zuzana, Čapková Linda, Kodet

TCR gamma/delta LGL proliferation causing recurrent episodes of neutropenia proceeding into fatal hepatosplenic T-cell lymphoma in an adolescent girl 5 years after renal transplantation .

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Mejstříková Ester, Sumerauer David, Froňková Eva, Mužíková Kateřina, Seeman Tomáš, Zemanová Zuzana, Čapková Linda, Kodet

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  1. TCR gamma/delta LGL proliferation causing recurrent episodes of neutropenia proceeding into fatal hepatosplenic T-cell lymphoma in an adolescent girl 5 years after renal transplantation Mejstříková Ester, Sumerauer David, Froňková Eva, Mužíková Kateřina, Seeman Tomáš, Zemanová Zuzana, Čapková Linda, Kodet Roman, Edita Kabíčková, Ondřej Hrušák Departments of Pediatric Hematology and Oncology, Pediatrics, Pathology 2nd Faculty of Medicine, University Hospital Motol Center of Tumor Cytogenetics, First Faculty of Medicine, General University Hospital Charles University Prague

  2. 24 22 20 18 16 absolute neutrophil count 14 12 10 8 6 4 2 0 10/2006 neutropenia, marrow aspirate normal, stomatitis january - february 2007 atypical "monocytes" in blood 10/2006 - january 2007 02/2005 – 10/2006 16-years old female 5-years after kidney transplantation for end stage renal failure (Fanconi nephronopthisis) combined immunosuppression (prednisone, tacrolimus, mycophenolate) time

  3. Immunophenotype of "atypical monocytes" CD7dimCD5negTCRgamma/delta+CD3bright+ CD7 Forward scatter CD5 CD3 Side scatter Side scatter proven clonality in TCR genes VgI-JG1.3 Dd2-Jd1-3 cytogenetic including FISH no abnormality

  4. Other findings • transient hepatosplenomegaly by ultrasound • anemia, transient trombocytopenia • increased triacylglycerols (up to14.28 mmol/L 4.2.2007, ferritin not analysed) • retrospectivelly extremely increased sCD25 (incipient organ rejection, marker of T cell proliferations) Interim diagnosis Proliferation of clonal atypical T cells morphologically appearing as monocytes = previously described posttransplant non malignant chronic condition (Tαβ more frequent) Treatment started 6- mercaptopurin, continued G-CSF

  5. May 2007, 3 months after beginning of treatment with 6-mercaptopurin Since 5/2007 febrile, pancytopenia, abdominal distension (ascites, organomegaly). WBC: Leu 6.0, Ery 2.29, Hb 70, hct 0.205, tr 10, atypical cells 62%, LDH (IFCC) 27.02, ferritin 10647 FACS (blood): same immunophenotype as in January, increased SG2M fraction (>20%) in bone marrow and peripheral blood

  6. Change of clinical behaviour = hepatosplenic T– NHL what‘s known: rare type of extranodal lymphoma young adults (median age, 35 years) typical B-symptoms, organomegaly splenomegaly + hepatomegaly, lymphadenopathy usually not present often pancytopenia also identified in patients treated with immunosuppression (after organ transplants or after TNF-alfa inhibitors)

  7. VCR 1.5 mg/m2 Daunorubicin 30 mg/m2 Asparaginase 10 000 U/m2 Fludarabin 30 mg/m2/d1-3 MabCampath 3,10,3x30mg Prednisone 60 mg/m2/d Therapy (day 1-13) WBC x 109 /l WBCx 109 /L days of therapy no response, death 13 days since diagnosis

  8. Change of biological behaviour • cytogenetics 6/2007 – newly identified isochromosome 7, abnormality often identified with hepatosplenic lymphoma

  9. Retrospective analysis of clonal atypical T cells neutropenia march 06 normal WBC

  10. Conclusions • prelymphoma cells found in patient more than 1 year before lymphoma manifestation • Isochromosome found as a late change during the lymphomagenesis • hepatosplenic lymphoma after solid organ transplantation: very rare, very poor outcome, no therapeutic approach established

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