A case of hairy cell leukemia variant

Vol. 56 No. 2 Suppl., 2015
This supplement was not sponsored by Outside Organizations.


Amelia Maria Gaman, Camelia-Marioara Dobrea, Mihnea-Alexandru Gaman

Hairy cell leukemia variant (HCLv) is a rare B-cell chronic lymphoproliferative disorder with features of the classic HCL but presenting some particularities, a poor response to conventional therapy of classic HCL and a more aggressive course of disease with shorter survival than classic HCL. We present a case of a 52-year-old man hospitalized in July 2012 in the Clinic of Hematology of Craiova, Romania, having splenomegaly, leukocytosis with lymphocytosis, anemia and thrombocytopenia, without monocytopenia, which exposed, in the peripheral blood and bone marrow cells, intermediate morphology between hairy cells and prolymphocytes and immunophenotype of mature B-cell phenotype CD19, CD20, CD22, CD11c, CD103, low positive for CD25 and negative for CD3, diagnosed with HCL variant, with no response to conventional chemotherapy and interferon-alpha, an aggressive course of disease and a survival of less than a year from diagnosis.

Corresponding author: Amelia Maria Gaman, Professor, MD, PhD; e-mail: gamanamelia@yahoo.com

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Luminita Paduraru, Daniela Claudia Scripcaru, Gabriela-Ildiko Zonda, Andreea-Luciana Avasiloaiei, Maria Stamatin

Teratoma is one of the most frequent fetal intracranial tumors, but it usually grows very quickly and the fetus is generally a stillborn. Rare cases have slow development or are located in areas that afford immediate surgery after birth with variable chances of survival. Even more rare cases survive days or weeks, but with no chance of surgical treatment and with prolonged palliative care. We present a 34 weeks premature infant, born by C-section with a giant intracranial tumor, whose origin could not be ascertained, occupied almost all-intracranial space and survived 25 days with supportive care. The histological examination established a G3 mixed teratoma, predominantly with immature cells from all three embryonic layers. The cerebellum was normal and infra-mesencephalic structures were present. The infant presented with severe anemia and mild respiratory distress, and was out of neurosurgical therapeutic resources. Antenatal examination was normal until 30 weeks, when fetal ultrasound described a degree of hydrocephalus, but no tumor was individualized. Conclusions: G3 type complex teratoma, even rare, can be localized at cerebral level and get giant development and growth only in the third trimester of pregnancy, ending with a neonate that has no chance of survival. Such cases cannot benefit of therapeutic interruption of pregnancy and generate serious difficulties for parents and clinicians.

Corresponding author: Luminita Paduraru, Lecturer, MD, PhD; e-mail: luminita.paduraru@gmail.com

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