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

Öz

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.

Anahtar Kelimeler

• 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.

Kaynakça

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