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Starry sky

Starry sky. Nicolas ORTONNE Assistance Publique des hôpitaux de Paris, Department of Pathology, Henri Mondor hospital, Créteil, France nicolas.ortonne@aphp.fr. Medical history. 72 years-old-female Auricular fibrillation, hypertension, depression, rheumatoid arthritis

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Starry sky

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  1. Starry sky Nicolas ORTONNE Assistance Publique des hôpitaux de Paris, Department of Pathology, Henri Mondor hospital, Créteil, France nicolas.ortonne@aphp.fr

  2. Medical history • 72 years-old-female • Auricular fibrillation, hypertension, depression, rheumatoid arthritis • Current treatments : vitamin D3, Duloxetin, fluindione, Ramipril, Cordarone, beta blocker, oral steroids (prednisone), iron spupplemntation, esomeprazole • Large necrotic mass on the left arm growing since 1 month. • Bad general condition since one year : night sweats, asthenia, weight loss • CT scan : homogeneous mass in the left deltoid muscle (18 x 9 x 7 cm). No underlying bone involvement. • Blood transfusion before admission in our emergency care unit.

  3. Laboratory • Leucocytes : 7.9 Giga/l, Hb: 8.3 g/dl, VGM 93 fl, Platelets : 90 Giga/l • Alkalin Phospatase : 72 UI/l, ALAT 18 U/l, ASAT *52, g GT : *115 UI/l, total bilirubin : *54.0 µmol/l, conjugated bilirubin : *30.0 µmol/l • raised LDH *1350 UI/l • CRP : *76.3 mg/l • Uric acid : 353 µmol/l • EBV viral load >10000000 copies/ml (>7 log) • Negative HVB, HCV, HIV, HTLV1 serologic tests

  4. Clinical diagnostic hypotheses? • Primary or secondary cutaneous carcinoma • Primary cutaneous lymphoma of B, T or NK origin • Secondary involvement by lymphoma/leukemia • Cutaneous angiosarcoma

  5. Skin biopsy

  6. CD20

  7. CD3

  8. BCL6

  9. CD10

  10. BCL2 : negative • MUM1 : negative • BCL1 / cyclin D1 : négative CD10

  11. Ki-67

  12. C-Myc cMYC

  13. EBV (EBER ISH)

  14. FISH studies : • MYCrearrangement • No BCL2/BCL6 rearrangements

  15. Diagnosis? Cutaneous localization of Burkitt’s lymphoma (sporadic form)

  16. Outcome • Node biopsy : same aspect • Bone marrow biopsy : normal • Polychemotherapy : COP for debulking then Rituximab and mini CHOP  partial response • Severe infection (urinary and pulmonary origin) • Death one month after diagnosis

  17. Burkitt’s lymphoma • Agressive large B-cell lymphoma • MYC gene rearrangement • 3 variants : 1/ Endemic: - Africa (equatorial), mainly children : 4-7 years-old - EBV often positive - Jaws +++ 2/ Sporadic: 1 à 2% of adult’s lymphomas in Europe and US 3/ Immunodepression (HIV): extranodal forms +++

  18. Burkitt’s lymphomas in adults • 2 variants : - Classical Burkitt’s lymphoma (BL) : rare but possible - Burkitt-like lymphoma (BLL)

  19. Burkitt’s lymphomas in the skin… • Very rare • Only isolated case reports, mostly in HIV+ patients • Corti M, et al. Non Hodgkin’s lymphoma with cutaneous involvement in AIDS patients: report of five cases and review of the literature. Braz J Infect Dis. 2010;14(1):81ı5. • Berk DR,et al. Burkitt lymphoma with cutaneous involvement. Dermatol Online J. 2008;14(2):14. • Jacobson MA, et al. Cutaneous involvement by Burkitt lymphoma. J Am Acad Dermatol. 2006;54(6):1111ı3.

  20. MYC rearrangement is not a specific feature of BL… • MYC rearrangement may be found in a subset of primary cutaneous BCL leg type Pham-Ledard A, et al. Modern Pathol 2013; 156: 1-10

  21. In conclusion • BL rarely but sometimes involve the skin. • Usually a secondary localization but may reveal the disease • « Starry sky » and monomorphous morphology • Centrogerminative phenotype without BCL2 and MUM1 expression • Prognostic significance of skin involvement : too rare to conclude…? • Prognostic significance of MYC rearrangement in PCBCL leg type?

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