Ritcher transformation, CLL transforms to
Correct Answer: Diffuse large B-cell lymphoma
Description: Richter's Syndrome (RS), also known as Richter's Transformation, is a rare complication of Chronic Lymphocytic Leukaemia (CLL) and/or Small Lymphocytic Lymphoma (SLL). It is characterised by the sudden transformation of the CLL/SLL into a significantly more aggressive form of large cell lymphoma. Richter's Syndrome occurs in approximately 2-10% of all CLL/SLL patients during the course of their disease. In the most cases the normally slow growing, or indolent, CLL transforms into a common type of non-Hodgkin lymphoma (NHL) known as Diffuse Large B-Cell Lymphoma (DLBCL). Rarer cases transform into Hodgkin lymphoma(HL)/Hodgkin Disease (HD), and some types of T-cell lymphomas also have been repoed The most common symptoms of Richter's Syndrome arise from a sudden and dramatic increase in the size of lymph nodes (also known as 'lymphadenopathy') characterised by usually painless areas of swelling in the neck, axilla, abdomen (most commonly in the spleen, also known as 'splenomegaly') or groin. Patients also often experience a dramatic increase in unexplained weight loss, fevers and night sweats (often collectively referred to as 'B-symptoms'). Other signs and symptoms may include low haemoglobin (anaemia) characterised by: increasing fatigue shoness of breath dizziness and possible palpitations low platelets (thrombocytopaenia) characterised by excessive bruising or bleeding an increase in serum lactate dehydrogenase (LDH) or elevated serum calcium (hypercalcaemia) on routine blood tests. It is impoant to note that whilst some of these signs and symptoms may already be present to a lesser degree in people with previously diagnosed CLL/SLL, the possibility of Richter's Syndrome needs to be considered if there is a sudden and dramatic increase in these symptoms, which is not generally considered to be a typical feature of normally relatively indolent CLL/SLL disease. Richter's syndrome is a serious complication of CLL/SLL and unfounately is often fatal. If Richter's syndrome is diagnosed, it is advised that the person should seek recommended treatments, but also to get their lives in order to prepare for any outcome. You may wish to discuss an advanced health directive with your treatment team, to let them and your family know how you would like your health to be managed if your treatments are not successful Richter's Syndrome affects approximately 2-10% of patients with a prior diagnosis of CLL/SLL and can occur at any time throughout the course of the disease. Ref Davidson 23rd edition pg 789
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