Nadir bir vaka: Spontan tibia kırığı ile presente olan Richter transformasyonu

Abstract

Richter sendromu (RS), kronik lenfositik lösemi/küçük lenfositik lenfomanın agresif lenfoid maligniteye dönüşmesi olarak tanımlanır. RS insidansı %1 ile %9 arasında değişir ve genellikle agresif bir klinik seyir gösterir. Sunulan olgu, KLL tanısı ile izlenen ve kemik tutulumu ile atipik şekilde presente olan RS vakasıdır. RS, düşük tedavi yanıt oranları ve kötü sağkalım ile karakterizedir. Esas olarak lenf nodu ve kemik iliği tutulur ancak vakamızdaki gibi ekstra nodal tutulumlar da görülebilir. Hastamız kemik tutulumu olan ilk RS olgusudur.

Keywords

• Kemik lezyonu
• Kronik lenfositik lösemi
• lenfoma
• Ritchter sendromu

A rare entity: Richter transformation presented with spontaneous tibia fracture

Abstract

Richter syndrome (RS) is defined as the transformation of chronic lymphocytic leukemia (CLL) /small lymphocytic lymphoma into an aggressive lymphoid malignancy. The incidence of RS varies from 1% to 9% and it is generally characterized by an aggressive clinical course. The case is here reported of CLL patient with RS and its unique presentation with bone involvement. RS is a heterogeneus condition that is characterised by an aggressive presentation, with low treatment response rates and and very poor survival. Lymph node and bone marrow are mainly involved but extra-nodal involvement is also observed. Our patient is the first case of RS with bone involvement.

References

1. 1. Hallek, M., Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment. American journal of hematology, 2017. 92(9): p. 946-965.
2. 2. Richter, M.N., Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. The American journal of pathology, 1928. 4(4): p. 285.
3. 3. Molica, S., A systematic review on Richter syndrome: what is the published evidence? Leukemia & lymphoma, 2010. 51(3): p. 415-421.
4. 4. Rossi, D. and G. Gaidano, Richter syndrome. Adv Exp Med Biol, 2013. 792: p. 173-91.
5. 5. Seymour, J. and J. Campbell, Richter's syndrome. BASIC AND CLINICAL ONCOLOGY, 2001. 26: p. 459-484.
6. 6. Omoti, C.E. and A.E. Omoti, Richter syndrome: a review of clinical, ocular, neurological and other manifestations. British journal of haematology, 2008. 142(5): p. 709-716.
7. 7. Rossi, D., et al., Biological and clinical risk factors of chronic lymphocytic leukaemia transformation to Richter syndrome. British journal of haematology, 2008. 142(2): p. 202-215.
8. 8. Parikh, S.A. and T.D. Shanafelt, Risk factors for Richter syndrome in chronic lymphocytic leukemia. Current hematologic malignancy reports, 2014. 9(3): p. 294-299.

9. 9. Parikh, S.A., et al., Diffuse large B‐cell lymphoma (R ichter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients. British journal of haematology, 2013. 162(6): p. 774-782.
10. 10. Falchi, L., et al., Correlation between FDG/PET, histology, characteristics, and survival in 332 patients with chronic lymphoid leukemia. Blood, 2014. 123(18): p. 2783-2790.
11. 11. Tadmor, T., et al., Richter's transformation to diffuse large B‐cell lymphoma: A retrospective study reporting clinical data, outcome, and the benefit of adding rituximab to chemotherapy, from the I sraeli CLL S tudy G roup. American journal of hematology, 2014. 89(11): p. E218-E222.
12. 12. Jenke, P., et al., Cyclophosphamide, adriamycin, vincristine and prednisone plus rituximab (CHOP-R) in fludarabine (F) refractory chronic lymphocytic leukemia (CLL) or CLL with autoimmune cytopenia (AIC) or Richter's transformation (RT): final analysis of a phase II study of the German CLL Study Group. 2011, Am Soc Hematology.
13. 13. Eyre, T.A. and A. Schuh, An update for Richter syndrome–new directions and developments. British journal of haematology, 2017. 178(4): p. 508-520.
14. 14. Rossi, D., et al., The genetics of Richter syndrome reveals disease heterogeneity and predicts survival after transformation. Blood, 2011. 117(12): p. 3391-3401.