Small Cell non-Hodgkin's Lymphoma Research Articles

RARE-29. PRIMARY CENTRAL NERVOUS SYSTEM NON-HODGKIN LYMPHOMA IN AN 11-YEAR-OLD BOY: A CASE REPORT

BACKGROUNDPrimary central nervous system lymphoma (PCNSL) are very rare in children. CLINICAL CASE: An 11-year-old male presented with a 2 months history with myoclonic movements in the upper right limb, and a sudden frontal headache, gait disturbance due to right hemiparesis and an ipsilateral convulsive episode. Upon admission he had critical condition, with hypertensive skull syndrome, Glasgow of 12, Karnofsky 40%, right hemiparesis, swallowing disorder, facial paralysis, and loss of photo motor reflex and unilateral amaurosis. A CT and MRI showed a huge tumor mass in the left tempo-parietal region, infiltrating the white matter and shifting the midline. A Tumor biopsy was done, and reported diffuse small cell non-Hodgkin lymphoma of high-grade, Burkitt type. Systemic lymphoma workup was negative. He received six cycles of chemotherapy based on high dose methotrexate, rituximab and triple intrathecal.After the second cycle an ophthalmologic evaluation was done, and found infiltration to the right retina, for which 6 cycles of intra vitreous chemotherapy with methotrexate were applied, he showed an excellent response, and recovered all his neurological functions except that right hemianopia persist. Control MRI showed partial response at 2nd cycle and complete response after the 4th cycle. No Radiation was performed.CONCLUSIONThis report highlights the fact that pediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy without irradiation. Lack of awareness of this rare entity may lead to extense resections of brain, and potential permanent secuelae that were avoided in this illustrative case.

Splenectomy in Lymphoproliferative Disorders: A Single Eastern European Center Experience.

Background and Objectives: Hematological malignancies are usually systemic diseases of life-threatening impact, and frequently require prompt and energetic therapeutic intervention. Due to systemic involvement, the role of surgery is generally limited to diagnostic approaches, and it is very rarely employed as a therapeutic modality. Splenectomy represents an exception to this paradigm, being used both as a diagnostic and tumor debulking procedure, notably in splenic lymphomas. Materials and Methods: We investigated the role of splenectomy in a single center prospective study of splenectomy outcome in patients with splenic involvement in the course of lymphoproliferative disorders. In the present study, we included all patients treated in our department for lymphoid malignancies over a period of six years, who underwent splenectomy as a diagnostic or debulking procedure after referral and workup, or had been referred to our department after first being splenectomized and diagnosed with splenic lymphoma. Patient characteristics and treatment outcome were investigated. Results: We enrolled 54 patients, with 34 (63%) splenectomized patients: 12 splenectomies (22.2%) for diagnostic purposes and 22 (40.7%) for treatment. Special attention was given to the 28 (51.85%) patients diagnosed with splenic marginal zone lymphoma (SMZL), a subtype with a clear therapeutic indication for splenectomy. Average age of patients was 57.5 (±13.1) years with a higher prevalence of feminine gender (66.67%). Age above 60 years old (p = 0.0295), ECOG (Eastern Cooperative Oncology Group) > 2 (p = 0.0402) and B-signs (p nonsignificant (NS)) were most frequently found in SMZL patients. Anemia, and notably autoimmune anemia, was more frequent in SMZL versus other small-cell lymphomas and also in splenectomized patients, as was leukocytosis and lymphocytosis. Treatment of patients with lymphoproliferative disorders consisted of chemotherapy and/or splenectomy. Most SMZL patients received chemotherapy as first line treatment (61.5%) and had only partial response (57.7%). Second treatment line was splenectomy in 80% of patients who required treatment, followed by a 60% rate of complete response (CR). Splenectomy offered a higher complete response rate (twice as high than in non-splenectomized, regardless of histology type, p = NS), followed by a survival advantage (Overall Survival (OS)~64 versus 59 months, p = NS). Particularly, SMZL patients had a 4.8 times higher rate of CR than other non-Hodgkin lymphoma (NHL) patients (p = 0.04), a longer progression free survival (73 months vs. 31 months for other small-cell NHLs p = NS) and a 1.5fold lower death rate (p = NS). The procedure was rather safe, with a 38.5% frequency of adverse reactions, mostly minor and manageable. Conclusions: Our data suggest that splenectomy is an effective and safe therapeutic option in patients with lymphoid malignancies and splenic involvement, particularly splenic marginal zone lymphoma.

Metastatic non-Hodgkin lymphoma presenting as low back pain and radiculopathy: a case report

ObjectiveThe purpose of this case report is to describe a case of metastatic non-Hodgkin lymphoma in the lumbar spine presenting as lumbar radiculopathy. Clinical FeaturesA 46-year-old man sought care from his doctor of chiropractic for low back pain and right leg radiculopathy. The patient was referred for a magnetic resonance imaging (MRI) scan to evaluate for a suspected disk herniation. The MRI scan revealed 2 lumbar pathologic compression fractures with cauda equina compression, and MRI short tau inversion recovery (STIR) sagittal image of the lumbar spine showed high signal in T12 and S2. Intervention and OutcomeThe patient was referred for an immediate consultation with his medical physician with the preliminary diagnosis of metastatic bone lesions or primary bone lesions of unknown etiology. The patient underwent bone biopsy, computed tomography, and positron emission tomography scanning and was diagnosed with small cell non-Hodgkin lymphoma with osseous metastasis. The patient underwent chemo- and radiation therapy, and the lymphoma is now in remission 18 months later. ConclusionThis case describes the presentation of metastatic non-Hodgkin lymphoma as a possible contributing cause in a patient presenting with lumbar radiculopathy, a common musculoskeletal condition. As well, this case highlights the importance of STIR sequences as part of a routine lumbar spine MRI examination. Without the STIR sequences, the additional deposits in T12 and S1 would not have been readily appreciated. Although metastatic non-Hodgkin lymphoma of the spine is rare, it should be remembered in the differential diagnoses.

The utility of interphase fluorescence in situ hybridization for the detection of the translocation t(11;14)(q13;q32) in the diagnosis of mantle cell lymphoma on fine-needle aspiration specimens

Mantle cell lymphoma can be difficult to differentiate cytologically from other small cell non-Hodgkin lymphomas. Nevertheless, the distinction is important, because mantle cell lymphoma is more aggressive than other small cell non-Hodgkin lymphomas. The purpose of this study was to determine whether fluorescence in situ hybridization (FISH) is helpful in diagnosing mantle cell lymphoma on fine-needle aspiration (FNA) specimens by detecting the t(11;14)(q13;q32) translocation that is characteristic of this tumor. Fifty-five lymph node FNA specimens from 53 patients were analyzed using FISH. A 2-color FISH assay that employed probes at the 14q32 (immunoglobulin H) and 11q13 (dual-colored, directly labeled cyclin D1) loci was used. The number of single-fusion and double-fusion signals in 200 cells was counted. If > or = 14% single-fusion signals or > or = 1.5% double-fusion signals or both were present, then the sample was considered FISH positive. The findings were correlated with the cytologic, histologic, and immunophenotypic findings in each specimen. Of the 55 cytology specimens, 17 were mantle cell lymphomas, and 38 were nonmantle cell lymphomas, including 16 small lymphocytic lymphomas (9 of 16 in an accelerated phase), 5 large cell lymphomas, 5 follicular lymphomas, 7 transformed large cell lymphomas (Richter syndrome), 3 atypical lymphoid proliferations, and 2 low-grade B-cell lymphomas. All 17 mantle cell lymphomas were positive by FISH. In addition, there were six small lymphocytic lymphomas (two in accelerated phase), one transformed large cell lymphoma, and one large cell lymphoma of follicular origin positive by FISH. The mean number of single-fusion and double-fusion signals, respectively, was 36 and 33 in mantle cell lymphoma specimens and 19 and 3 in positive nonmantle cell lymphoma specimens. The detection of the t(11;14)(q13;q32) translocation by FISH analysis was helpful in diagnosing mantle cell lymphoma on FNA specimens. Double-fusion signals were more specific for mantle cell lymphoma than single-fusion signals. In rare instances, other non-Hodgkin lymphomas also showed increased numbers of single-fusion signals that were not necessarily indicative of the t(11;14)(q13;q32) translocation. Therefore, in an initial diagnosis of mantle cell lymphoma, significant numbers of double-fusion FISH signals should be identified and interpreted in conjunction with the cytologic and immunologic studies.

Exfoliative respiratory cytology in the diagnosis of leukemias and lymphomas in the lung.

Leukemias and lymphomas involving the lung were diagnosed by means of exfoliative cytology in 31 specimens from 20 patients. Initial diagnostic categorizations included 29 specimens "positive for malignancy," including two thought to represent "carcinoma vs. lymphoma," and two considered suspicious for lymphoma. Previous diagnoses of lymphoma (13 patients) and acute myelogenous leukemia (AML) (2 patients) were available. In 5 additional patients, exfoliative respiratory cytology yielded the first diagnosis of hematopoietic malignancy. Cytologic diagnosis included nine large-cell and six small-cell non-Hodgkin's lymphomas (NHL), three Hodgkin's lymphomas (HD), and two AML. Key cytologic features included markedly pleomorphic and monomorphic cell populations in HD and NHL, respectively, as well as lack of tumor cell cohesion and necrosis in all cases. Cytologically, acute leukemia may be difficult to differentiate from large-cell NHL, and small-cell NHL from reactive/benign small lymphocytes. Blood, scant cellularity, crush artifacts, and apparent molding may affect diagnostic accuracy. Immunocytochemistry in cell block sections of sputa and washings is useful in the diagnostic workup in selected cases. Although involvement of the respiratory system by leukemias and lymphomas is uncommon and not always preceded by a history of malignancy, cytologic diagnosis is usually prompt, reliable, and accurate.

Exfoliative respiratory cytology in the diagnosis of leukemias and lymphomas in the lung

Leukemias and lymphomas involving the lung were diagnosed by means of exfoliative cytology in 31 specimens from 20 patients. Initial diagnostic categorizations included 29 specimens “positive for malignancy,” including two thought to represent “carcinoma vs. lymphoma,” and two considered suspicious for lymphoma. Previous diagnoses of lymphoma (13 patients) and acute myelogenous leukemia (AML) (2 patients) were available. In 5 additional patients, exfoliative respiratory cytology yielded the first diagnosis of hematopoietic malignancy. Cytologic diagnosis included nine large-cell and six small-cell non-Hodgkin's lymphomas (NHL), three Hodgkin's lymphomas (HD), and two AML. Key cytologic features included markedly pleomorphic and monomorphic cell populations in HD and NHL, respectively, as well as lack of tumor cell cohesion and necrosis in all cases. Cytologically, acute leukemia may be difficult to differentiate from large-cell NHL, and small-cell NHL from reactive/benign small lymphocytes. Blood, scant cellularity, crush artifacts, and apparent molding may affect diagnostic accuracy. Immunocytochemistry in cell block sections of sputa and washings is useful in the diagnostic workup in selected cases. Although involvement of the respiratory system by leukemias and lymphomas is uncommon and not always preceded by a history of malignancy, cytologic diagnosis is usually prompt, reliable, and accurate. Diagn Cytopathol 1996;14:108–113. © 1996 Wiley-Liss, Inc.

Bone marrow involvement by non-Hodgkin's lymphoma: the clinical significance of morphologic discordance between the lymph node and bone marrow. Nebraska Lymphoma Study Group.

Bone marrow specimens from 317 patients with non-Hodgkin's lymphoma (NHL) obtained at initial staging were evaluated for the presence of lymphoma or benign lymphoid aggregates. Thirty-two percent (102 patients) had lymphoma in their bone marrow, and 9% had benign lymphoid aggregates. Bone marrow lymphoma was present in 39% of low-grade, 36% of intermediate-grade, and 18% of high-grade lymphomas. The bone marrow was involved in 25% of patients with diffuse large-cell or immunoblastic NHL (ie, diffuse histiocytic lymphoma of Rappaport). Bone marrow involvement did not affect survival of patients with low-grade NHL, but survival was significantly shorter (P = .03) for patients with intermediate- and high-grade NHL with bone marrow involvement. Bone marrow involvement was equally common in B-cell and T-cell NHL (31% v 32%). However, patients with T-cell NHL and bone marrow involvement had shorter survival than B-cell NHL with marrow involvement (P = .02) or T-cell NHL without marrow involvement (P = .05). A high incidence of morphologic discordance between lymph node and bone marrow was observed (ie, 40%), always with a more aggressive subtype in the lymph node than in the bone marrow. Presence of large-cell lymphoma in the bone marrow predicted for short survival. Survival for patients with small-cell lymphoma in their bone marrow did not differ significantly from patients with negative bone marrows. We conclude that bone marrow involvement in large-cell NHL, especially in those of T-cell origin, portends a poor prognosis. However, the subgroup of patients with an aggressive histologic subtype of NHL in a lymph node biopsy and small-cell NHL in the bone marrow do not have a poorer outlook than those without bone marrow involvement.