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Year : 2015  |  Volume : 52  |  Issue : 3  |  Page : 329-330

Isolated Richter's transformation of brain parenchyma: Remission with DeAngelis protocol

1 Department of Medical Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication18-Feb-2016

Correspondence Address:
V Raina
Department of Medical Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.176695

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How to cite this article:
Gogia A, Iqbal N, Sharma M C, Raina V. Isolated Richter's transformation of brain parenchyma: Remission with DeAngelis protocol. Indian J Cancer 2015;52:329-30

How to cite this URL:
Gogia A, Iqbal N, Sharma M C, Raina V. Isolated Richter's transformation of brain parenchyma: Remission with DeAngelis protocol. Indian J Cancer [serial online] 2015 [cited 2020 Jul 5];52:329-30. Available from:


Richter's syndrome (RS) is transformation of chronic lymphocytic leukemia (CLL) to a diffuse large B cell lymphoma (DLBCL), which can occur up to 5% of patients with CLL.[1] The clinical outcome of RS is poor and the median survival ranges from 6 to 8 months. RS is usually characterized by systemic symptoms (fever, weight loss and/or drenching night sweats) with the rapid increase in the size of lymphoid organ.[2] Isolated intracranial RS is rare.

A 56-year-old male patient referred to our hospital in June 2002 with symptoms of fatigue and generalized lymphadenopathy. Clinical examination revealedEastern Cooperative Oncology Group performance status-1, mild pallor, generalized lymphadenopathy and splenomegaly. His hemogram showed hemoglobin - 10.5 g/dL, total leucocyte count - 50 × 109/L and platelet counts-110 × 109/L. Peripheral blood smear examination showed normochromic normocytic anemia with 80% lymphoid cell. Flow cytometric analysis of peripheral blood revealed 80% CD19+, CD5+ abnormal lymphoid cell, which are positive of CD20, CD23, negative for FMC 7 and CD 3. Renal and liver functions were within the normal limit. Direct Coomb's text was negative and there are no features of hemolysis. Diagnosis of CLL Rai Stage III was made and the patient was started fludarabine (25 mg/m 2) d1-3, cyclophosphamide d1-3 (250 mg/m 2) each over 28 days. After six cycles, the disease was in complete remission. He again presented in July 2012 with complaints of headaches and disorientation. Physical examination revealed left sided hemiparesis with facial nerve involvement. Contrast-enhanced computed tomography (CECT) scan of the brain revealed two well-defined lesions in the right temporal and occipital lobe. Positron emission tomography-computed tomography whole body was normal except abnormal uptake in right temporal and occipital lobe [Figure 1]a and [Figure 1]b. Brain biopsy from lesion indicated high grade DLBCL post germinal cell type positive for CD45, CD20, BCL-6 and MUM-1 and negative for CD10 and Cyclin-D1 [Figure 1]c and [Figure 1]d. Hemogram and biochemistry profile was normal. Serum lactate dehydrogenase was 329 U/L. Peripheral smear, bone marrow examination and cerebrospinal fluid examination was normal. His human immunodeficiency virus serology was negative. He was started on specific chemotherapy protocol for central nervous system (CNS) lymphoma, DeAngelis protocol comprising of high dose methotrexate, intrathecal methotrexate, vincristine, procarbazine, dexamethasone, high dose cytarabine with whole brain radiotherapy (WBRT).[2] In post three cycles of chemotherapy, there was a marked improvement in his symptoms with complete resolution of his facial palsy and hemiparesis with the normal orientation. CECT scan brain showed a significant reduction in the size of lesions.
Figure 1: (a and b) Positron emission tomography-computed tomography scan showing well-defined mass lesions in right temporal and occipital area with an increased fluorodeoxyglucose uptake; (c) Brain biopsy specimen showing diffuse lymphoid replacement of uniform large, vesicular tumor nuclei with moderately abundant cytoplasm (H and E, ×200); (d) The tumor cells show diffuse strong membranous expression of CD20 by immunohistochemistry (×200)

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RS may be found in the brain as a primary location with or without systemic evidence of transformation. CNS involvement can be isolated meningeal involvement, brain parenchymal lesion or in combination. Few cases have been reported of RS of the brain.[3],[4],[5] Among these, diagnosis was established simultaneously with CLL in five cases, isolated leptomeningeal involvement in five cases and four cases were similar to our case, i.e., isolated brain parenchymal lesion. All the reported cases were treated WBRT. We treated our case with chemotherapy employing drugs having good CNS penetration with DeAngelis protocol.[3] He responded well to treatment. As the prognosis of this disease is dismal, early recognition of symptoms, especially neurological symptoms in a patient of CLL with accurate histological diagnosis should lead to rapid institution of definitive therapy.

 » References Top

Tsimberidou AM, Keating MJ. Richter syndrome: Biology, incidence, and therapeutic strategies. Cancer 2005;103:216-28.  Back to cited text no. 1
DeAngelis LM, Seiferheld W, Schold SC, Fisher B, Schultz CJ, Radiation Therapy Oncology Group Study 93-10. Combination chemotherapy and radiotherapy for primary central nervous system lymphoma: Radiation Therapy Oncology Group Study 93-10. J Clin Oncol 2002;20:4643-8.  Back to cited text no. 2
Bagic A, Lupu VD, Kessler CM, Tornatore C. Isolated Richter's transformation of the brain. J Neurooncol 2007;83:325-8.  Back to cited text no. 3
Resende LS, Bacchi CE, Resende LA, Gabarra RC, Niéro-Melo L. Isolated Richter's syndrome in central nervous system: Case report. Arq Neuropsiquiatr 2005;63:530-1.  Back to cited text no. 4
Mahé B, Moreau P, Bonnemain B, Letortorec S, Menegali D, Bourdin S, et al. Isolated Richter's syndrome of the brain: Two recent cases. Nouv Rev Fr Hematol 1994;36:383-5.  Back to cited text no. 5


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