Transformación leucémica en paciente con linfoma folicular e hiperleucocitosis al diagnóstico / Leukemic transformation in a patient with follicular lymphoma and hyperleukocytosis at diagnosis

La fase leucémica como presentación de un linfoma folicular es rara y debe ser considerada factor de mal pronóstico. Por otra parte, la asociación entre linfoma folicular y síndrome mielodisplásico no se ha descrito. Se presenta el caso de una paciente en la que se detectó marcada leucocitosis y a la que se diagnosticó un linfoma folicular. Recibió quimioterapia con R-CHOP y FCR cuando recayó. Meses después, se realizó un aspirado medular en el cual se observaron cambios compatibles con mielodisplasia, únicamente recibió terapia de soporte y finalmente evolucionó a leucemia mieloide aguda. Aunque se conoce que la mielodisplasia puede ser secundaria al uso de quimioterapia, la paciente presentó además trisomía del cromosoma 11, descrita previamente en mielodisplasia y linfoma tipo Burkitt, la cual pudiera estar en relación con la evolución a leucemia mieloide aguda(AU)

Follicular lymphoma rarely presents with a leukemic phase and this should be considered a negative prognostic factor. Also, follicular lymphoma and myelodysplastic syndrome association has not been previously reported. Herein we present a patient who debuted with marked hyperleukocytosis and was diagnosed with follicular lymphoma, receiving CHOP-R and FCR after she relapsed. Several months later, secondary myelodysplastic changes were observed in her bone marrow. She received supportive therapy and finally progressed into acute myeloid leukemia. Although secondary myelodysplasia is known to be produced by chemotherapy, this patient additionally had trisomy 11, previously described in myelodysplasia and Burkitt's lymphoma, which could be linked to progression to acute myeloid leukemia(AU)

Leukemic phase of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) is a distinct type of CD30+ T/null-cell non-Hodgkin's lymphoma that frequently involves nodal and extranodal sites. The presence of leukemic phase in ALCL is extremely rare and occurs exclusively with ALK1-positive ALCL. We describe two patients with ALK1-positive ALCL who developed a leukemic phase with rapid progression of the disease. Immunophenotypic pattern assessed on peripheral blood by flow cytometry revealed CD45, CD30, and CD25 positivity in both cases but NPM-ALK1 was expressed in only one case. Both patients developed leukemic phase as a terminal event of the disease and we share the immunophenotypic features of both cases.

Clinico-hematologic Findings of Leukemic Bone Marrow Involvement in Korean Patients with Non-Hodgkin Lymphoma

BACKGROUND: The purpose of the present study was to investigate the clinico-hematological findings of bone marrow (BM) involvement and leukemic phase in Korean patients with non-Hodgkin lymphoma (NHL). METHODS: We included 791 patients with NHL that were classified with the WHO (2008) criteria. Laboratory data, bone marrow histomorphologic features and medical records were reviewed. Leukemic phase was defined as when the proportion of neoplastic lymphoid cells comprised more than 10% of leukocytes in the peripheral blood or more than 25% of nucleated cells in the BM. RESULTS: We found that 21.7% (172/791) of the patients had BM involvement, and 6.2% (49/791) developed leukemic phase of the disease. NHL subtypes showing high frequencies of leukemic phase development were mantle cell lymphoma (40%), angioimmunoblastic T cell lymphoma (40%), lymphoblastic lymphoma (36.4%), and Burkitt lymphoma (26.1%). Compared to B-cell type, T-cell type of NHL showed significantly higher frequencies of BM involvement (18.6% vs 30.9%; P=0.0004) and leukemic phase development (4.8% vs 10.3%, P=0.008). Complete remission rate was significantly lower in leukemic (55.6%) than in non-leukemic (85.9%) group of patients (P=0.0002), whereas relapse rate was not different between the two groups. Death rate was higher in leukemic (46.9%) than in non-leukemic (30.1%) group of patients, and the 5-yr overall survival probability was significantly lower in leukemic group (P=0.02). CONCLUSIONS: The incidence of leukemic phase development in NHL was lower in Korean patients than that reported for Western populations and higher in T-cell lymphoma. We confirmed that the presence of leukemic phase in NHL patients is associated with a poor prognosis.

A Case of a Young Woman with Hepatosplenic gamadelta T-cell Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

Most T-cell lymphomas arise from mature alpabeta T-cells and commonly involve the nodes. Lymphomas bearing the gamadelta T-cell receptor (TCR) are very rare, and involve the lymph nodes minimally, if at all. Hepatosplenic gamadelta T-cell lymphoma is a recently identified, rare entity in which lymphoma cells bearing the gamadelta TCR infiltrate the sinusoids of the liver, splenic red pulp, and bone marrow. Its leukemic transformation is even more rare. Recently, we experienced a case of hepatosplenic gamadelta T-cell lymphoma in a 19-year-old woman who presented with epigastric pain, fever, massive splenomegaly, andpancytopenia. The splenectomy specimen and excisional biopsy of the liver revealed the infiltration of atypical T lymphocytes with the immunophenotypic markers of CD3 (+), CD45RO (pan-T antigen) (+), TIA-1(+), CD4(-),CD8 (-), CD56 (-), and S100 (-) in the sinusoids of the liver and splenic red pulp. Polymerase chain reaction (PCR) showed that these cells had the expression of the TCR gama gene rearrangements. Though the pancytopenia had improved after the splenectomy, the response of chemotherapy was transient. Her disease progressed rapidly and she expired in the leukemic phase. We report a case of hepatosplenic gamadelta T-cell lymphoma that developed in a young woman, along with a brief review of the literature.

Leukemic Phase of Mantle Cell Lymphoma, Blastic Variant / 대한혈액학회지

A case with mantle cell lymphoma, blastic variant, involving the peripheral blood in a 78- year-old female was described. The circulating blast-like cells suggested the possibility of acute leukemia. The WBC count was 28.0x109/ L with the absolute lymphoctyosis, 14.0x109/L. The peripheral blood smears showed a medium to large-sized lymphocytes with blast- like chromatin and conspicuous nucleoli. Atypical cells showing cleaved or indented nucleus were frequently observed. By flow cytometric analysis, immunophenotype of the circulating leukemic cells expressed B-cell associated antigens (CD 19, CD20), coexpressed CD5, lacked CD23, and expressed monoclonal surface immunoglobulin. Bone marrow aspiration and bone core biopsy confirmed diffuse infiltration of lymphoma cells. Immunohistochemicalstain for cyclin D1 (PRAD1/Bcl-1) protein was strongly reactive in the nucleus and cytoplasm of the infiltrative lymphoma cells in the bone marrow. Cytogenetic analysis of the bone marrow aspirates showed the complex abnormalities. Heterotransplantations of the blastic mantle cell leukemic cells, 4x104/microliter, into the peritoneum of the severe combined immunodeficiency (SCID) mouse showed tumor formation and infiltration of malignant lymphoid cells into peritoneum, liver, bone marrow, diaphragm, spinal cord and testes. The histopathology, immunophenotype and cyclin D1 protein expression of xenotransplanted tumor showed identical findings that of the original peripheral blood.