Annals of Hematology

, Volume 97, Issue 8, pp 1509–1511 | Cite as

Isolated Richter’s syndrome of the brain: diagnosis in the eye of the beholder

  • Ana Luísa Pinto
  • Gisela Ferreira
  • José Pedro Carda
  • Marília Gomes
  • Maria Letícia Ribeiro
Letter to the Editor

Dear Editor,

A 67-year-old woman presented with headache, diplopia, reduced bilateral visual acuity, and motor impairment on her right side. She had a diagnosis of high-count monoclonal B-lymphocytosis of chronic lymphocytic leukemia (CLL) for 3 years. Neurological examination revealed a complete left oculomotor palsy and right hemiparesis, and ophtalmological examination showed bilateral vitreous haze. Lymphadenopathy and hepatosplenomegaly were absent. Brain magnetic resonance imaging (MRI) showed a diffuse, gadolinium-enhancing lesion in the upper brainstem, thalamus, and internal capsule, predominantly on the left side (Fig. 1a, b). Cerebrospinal fluid analysis was negative for tumor cells. Brain biopsy revealed a focal infiltrate of a lymphoid neoplasia composed by medium-sized lymphocytes that expressed CD20/Bcl-2/Bcl-6 and high Ki-67 index. Reduced sample size hampered a definitive histopathological diagnosis. Given the risk of a second biopsy of the brainstem, a vitrectomy was...


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from the patient for being included in the study.


  1. 1.
    Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (2008) WHO Classification of tumours of haematopoietic and lymphoid tissues, 4th edn. IRAC, LyonGoogle Scholar
  2. 2.
    Jamroziak K, Tadmor T, Robak T, Polliack A (2015) Richter syndrome in chronic lymphocytic leukemia: updates on biology, clinical features and therapy. Leuk Lymphoma 56(7):1949–1958CrossRefPubMedGoogle Scholar
  3. 3.
    Fløisand Y, Delabie J, Fosså A, Helseth E, Jacobsen EA, Rolke J, Tjønnfjord GE (2011) Richter syndrome presenting as a solitary cerebellar tumor during first-line treatment for chronic lymphocytic leukemia. Leuk Lymphoma 52:2007–2009CrossRefPubMedGoogle Scholar
  4. 4.
    Ghofrani M, Tantiwongkosi B, Smith AS, Wasdahl DA (2007) Richter transformation of chronic lymphocytic leukemia presenting as a dural-based non-Hodgkin lymphoma mass. Am J Neuroradiol 28(2):318–320PubMedGoogle Scholar
  5. 5.
    Stuplich M, Mayer K, Kim Y, Thanendrarajan S, Simon M, Schafer N, Glas M, Schmidt-Wolf IGH (2012) Richter syndrome and brain involvement: low-grade lymphoma relapsing as cerebral high-grade lymphoma. Acta Haematol 127:93–95CrossRefPubMedGoogle Scholar
  6. 6.
    Ishida F, Nakazawa H, Takezawa Y, Matsuda K, Asano N, Sano K, Sakai H, Ito T (2013) Richter transformation in the brain from chronic lymphocytic leukemia. J Clin Exp Hematopathol 53(2):157–160CrossRefGoogle Scholar
  7. 7.
    Gogia A, Iqbal N, Sharma MC, Raina V (2015) Isolated Richter’s transformation of brain parenchyma: remission with DeAngelis protocol. Indian J Cancer 52(3):329–330CrossRefPubMedGoogle Scholar
  8. 8.
    Dunbar M, Dilli E, Slack GW, Villa D (2016) Chronic lymphocytic leukemia presenting with concurrent isolated CNS parenchymal and leptomeningeal large B-cell lymphoma. Ann Hematol Oncol 3(6):1098Google Scholar
  9. 9.
    Kroft SH, Dawson DB, McKenna RW (2001) Large cell lymphoma transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma: a flow cytometric analysis of seven cases. Am J Clin Pathol 115(3):385–395CrossRefPubMedGoogle Scholar
  10. 10.
    Woroniecka R, Rymkiewicz G, Grygalewicz B, Błachnio K, Rygier J, Jarmuz-Szymczak M, Ratajczak B, Pienkowska-Grela B (2015) Cytogenetic and flow cytometry evaluation of Richter syndrome reveals MYC, CDKN2A, IGH alterations with loss of CD52, CD62L and increase of CD71 antigen expression as the most frequent recurrent abnormalities. Am J Clin Pathol 143:25–35CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Ana Luísa Pinto
    • 1
  • Gisela Ferreira
    • 2
  • José Pedro Carda
    • 1
  • Marília Gomes
    • 1
  • Maria Letícia Ribeiro
    • 1
  1. 1.Clinical Hematology DepartmentCentro Hospitalar e Universitário de CoimbraCoimbraPortugal
  2. 2.Hematology DepartmentCentro Hospitalar do Baixo VougaAveiroPortugal

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