Subtype Of Large Cell Lymphoma Research Articles

From Tachycardia to Lymphoma: A Case Report of CD30 Positive (ALK) T-Cell Lymphoma Initially Presenting with Cardiac Envolvement

Anaplastic Large Cell Lymphoma (ALCL), Anaplastic Lymphoma Kinase-Positive (ALK+), is a rare subtype of large cell lymphoma. Cardiac involvement as the initial presentation leading to diagnosis is extremely rare. We present a young patient with atrial tachycardia as the presenting manifastation of ALCL.

A challenging case of intravascular large B-cell lymphoma presenting as pyrexia of unknown origin

Abstract Introduction: Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of large cell lymphoma, characterized by the growth of neoplastic cells within the lumina of small blood vessels, without an obvious extravascular tumor mass or presence of circulating lymphoma cells in the peripheral blood. Patient concern: A 68-year-old woman presented with fever of unknown origin and abdominal pain. Diagnosis: Bone marrow aspiration showed abnormally large cells with increased hemophagocytic activity. Trephine biopsy showed moderate to large neoplastic cells with single and multiple prominent nucleoli sequestered in the blood vessel lumen. Immunohistochemistry was positive for CD20, PAX5, CD79a, MUM1, and BCL2 and showed a high proliferative fraction of 80% confirming the diagnosis of IVLBCL. Interventions: The patient received six cycles of R-CHOP chemotherapy. Outcomes: The patient has been in remission for nearly two years after completing the treatment. Conclusion: Clinical recognition of IVLBCL remains a challenge. Standard staging and therapeutic approaches need to be addressed to further elucidate the characterization and management of this rare disease.

Intravascular large cell lymphoma mimicking central nervous system vasculitis in a patient with rheumatoid arthritis (RCD code: VI-2C.2)

Increased incidence of lymphomas in patients with rheumatoid arthritis has been reported and may be associated with several factors, including genetic factors, chronic inflammation, and immunosuppressive treatment. Intravascular large cell lymphoma (ILCL) is a rare subtype of large cell lymphoma with undetectable lymphoma cells in peripheral blood and without any extravascular localisation. Diag- nosis is often delayed because bone marrow, spleen, and cerebrovascular fluid may not be involved and there are no specific laboratory studies for intravascular large cell lymphoma. We report the case of a 63-year-old woman with a 10-year history of seropositive rheumatoid arthritis and intravascular large cell lymphoma recognised at autopsy. The patient presented with progressive central nervous system-related symptoms mimicking vasculitis without lymphadenopathy or bone marrow involvement. Malignant lymphoproliferative disorders should be considered in RA patients with varying clinical presentation, even in the absence of lymphadenopathy and bone marrow involvement. JRCD 2018; 4 (1): 15-17.

A Rare Case of Intravascular Large B-Cell Lymphoma

First reported in the literature in 1959, intravascular large B-cell lymphoma (IVLBCL) is a very rare subtype of large cell lymphoma characterized by the proliferation of clonal atypical lymphocytes within the lumina of small blood vessels, without any obvious extravascular tumor mass or readily observable circulating lymphoma cells in the peripheral blood. The surprising degree of sparing of the surrounding tissue and the absence of lymphoma cells in the lymph nodes and reticuloendothelial system are a hallmark of the disease. Here were present a case of a 63-year-old female, without any significant medical history, presenting with a macule on the left breast skin. A punch biopsy was performed, which on histology revealed atypical lymphoid cells (large cells with vesicular nuclear chromatin and prominent nucleoli) within the smaller blood vessels in the reticular dermis. There was no infiltration of these atypical cells in the surrounding dermis. With a strong suspicion of lymphoma, the first round of immunohistochemistry was performed, which showed negative results for CK7, CK20, mammaglobin, CD31, and CD68 and strong positivity for CK20, suggestive of IVLBCL. Second round of immunostains was positive for CD79a, MUM1, and BCL-2; CD10 was negative while CD3 showed admixed lymphocytes, supportive of the diagnosis. IVLBCL is a rare disease with an estimated incidence of less than one per million. Median age of incidence is 70 years (34–90 years). Patients present with a variety of constitutional and systemic symptoms (especially CNS involvement) caused by the occlusion of small vessels, but our patient did not present with any. Skin involvement can manifest as nodules/plaques (49%), macules (22.5%), telangiectatic patches (20%), and cellulitis. We present this rare and interesting case to highlight the existence of this entity since it carries a poor prognosis and requires a timely rituximab-containing chemotherapy plus CNS-oriented therapy.

Characteristics of T- and NK/T-cell lymphomas with different pathological subtypes on 18F-FDG PET/CT

Objective To investigate the imaging presentation of T- and NK/T-cell lymphomas with different pathology subtypes on 18F-FDG PET/CT. Methods A total of 95 patients (66 males, 29 females, average age 38.42 years) with T- and NK/T-cell lymphoma proved by pathology from June 2006 to February 2016 were retrospectively analyzed. 18F-FDG uptake (SUVmax), nodal invasion, nodal distribution, extra-nodal involvement and staging were compared among 7 pathological subtypes of T- and NK/T-cell lymphomas. One-way analysis of variance, Fisher exact test and Kruskal-Wallis H test were used for data analysis. Results There were significant differences in terms of 18F-FDG uptake, nodal invasion, nodal distribution, extra-nodal involvement and staging among different pathological subtypes of T- and NK/T-cell lymphomas (F=2.937, P<0.05; Fisher exact test, all P<0.01; H=19.883, P<0.01). NK/T-cell lymphoma was found to be prone to invade the nasal cavity and nasopharynx, enteropathic type T-cell lymphoma was specific to the intestine, and subcutaneous panniculitis-like T-cell lymphoma presented with subcutaneous infiltration. All those 3 subtypes were quite specific in their extra-nodal involvement. Most patients with angioimmunoblastic T-cell lymphoma (ATCL), peripheral unspecified T-cell lymphoma (PUTCL) and T immunoblastic lymphoma (TIBL) presented as stage Ⅳ disease. Widespread lymph node disease associated with splenic, parotid and serous membrane involvement were often seen in ATCL patients (most commonly to involve the parotid glands and serous membrane among the 7 subtypes). Nodal involvement was found in PUTCL patients, but extranodal involvement was rather non-specific. TIBL had a non-specific pattern of nodal involvement with low 18F-FDG uptake, lower than ATCL and the other 5 subtypes. Anaplastic large cell lymphoma subtypes had the highest 18F-FDG uptake when compared with the other 6 subtypes, but were less often to manifest as stage Ⅳ disease despite their preponderance for marrow and nodal infiltration. Conclusion Different pathological subtypes of T- and NK/T-cell lymphoma manifest different imaging presentations on 18F-FDG PET/CT, which are useful for understanding their biological characteristics. Key words: Lymphoma, T-cell; Natural killer T-cells; Positron-emission tomography; Tomography, X-ray computed; Deoxyglucose

Linfoma intravascular de células B grandes: hallazgos clínicos y morfológicos en un caso con desenlace fatal

Intravascular large B cell lymphoma is a rare subtype of large cell lymphoma that is characterized by the proliferation of lymphoid cells within the lumina of small blood vessels. We report a 61-year-old male presenting paresis of both lower limbs, confusion and a history of weight loss. Magnetic resonance and CAT imaging studies showed multiple images of brain and cerebellar infarctions. Twenty days after admission, the patient died and the postmortem study demonstrated a multisystem intravascular large B cell lymphoma.

Clinicopathological features of Epstein-Barr virus-positive diffuse large B-cell lymphoma in elderly

To investigate the clinicopathological features of EB virus positive diffuse large B-cell lymphomas (EBV + DLBCL) of the elderly. Four hundred and ninety-six cases of DLBCLs were retrospectively studied by in situ hybridization (ISH) to detect the EBV in tumor cells, and by immunohistochemistry to evaluate the expression of CD10, CD20, CD30, CD79a, bcl-6, bcl-2, MUM-1, CD5, CD3, TIA-1 and Ki-67 protein. Their clinicopathological correlations were analyzed. Of the 59 cases of EBV + DLBCL, 48 cases were EBV positive. The median age of these EBV + DLBCLs was 73 years with male predominance (1.4:1). There were 11 cases with nodal presentation only, 18 cases with extra-nodal presentation and 19 cases with both lymph nodal and extra-nodal involvements, whereas about one third cases with more than one extra-nodal involvement. Thirty-five patients presented with advanced disease (Ann Arbor stage III/IV). A performance status was available in 36 cases and 5 cases had performance status of more than 1. Seven of 30 patients were found with high lactate dehydrogenase value (more than twice of the normal). An IPI-score was calculated in 30 cases and 18 cases had an intermediate/high IPI-score (3-5). The median survival for these patients was 35 months. Morphologically, EBV + DLBCLs of the elderly generally showed a diffuse and polymorphic proliferation of large lymphoid cells with varying degrees of reactive components including small lymphocytes, plasma cells, histiocytes, and epithelioid cells. These tumor cells were frequently characterized by a broad range of B-cell maturation, containing centroblasts, immunoblasts, and Hodgkin- and Reed-Sternberg (HRS)-like giant cells. The study cohort was further morphologically divided into large cell lymphoma subtypes (n = 33) and polymorphic lymphoma subtypes (n = 14) and one case with mixed subtype. Immunohistochemical studies showed that tumor cells were positive for CD20 (47/48) and/or CD79a (45/45) in almost cases. Tumor cells were MUM-1-positive in the majority of the cases (44/47) and were stained for CD10 or bcl-6 in a few cases. Expression of bcl-2 and CD30 was observed in 80.0% (28/35) and 28.9% (11/38) cases, respectively, and most of the cases (33/39) had a high proliferative index (by Ki-67 with a 50% cut-off point). Compared with other EBV + DLBCLs, except the older age and low frequency of bcl-6 staining, no other significant differences were observed in EBV + DLBCLs of the elderly. EBV + DLBCLs of the elderly constitute a distinct clinicopathologic subtype of DLBCL, although many clinical and histological features with EBV + lymphomas are similar with that of younger ages. Differential diagnosis from other types of lymphomas should also be considered.

Senile EBV+ B-cell lymphoproliferative disorders: a clinicopathologic study of 22 patients.

Twenty-two Epstein-Barr virus-associated B-cell lymphoproliferative disorders (LPDs) without predisposing immunodeficiencies were evaluated clinically and pathologically. All patients were Japanese and negative for anti-human immunodeficiency virus antibody. They were all more than 60 years old with a median age of 75.5 years. Eighteen (82%) patients showed extranodal involvement. Biopsied specimens contained variable numbers of centroblasts, immunoblasts, and Reed-Sternberg-like giant cells often with necrosis and an angiocentric pattern. The 13 cases showing polymorphous composition and inflammatory background were categorized as polymorphic LPD subtype. The other nine cases contained diffuse proliferative lesions of large lymphoid cells and were categorized as large cell lymphoma subtype. Tumor cells expressed CD20 and/or CD79a, and in situ hybridization showed them to be associated with Epstein-Barr virus. LMP1 was detected in all cases and EBNA2 in seven. Eighteen patients initially received combination chemotherapy, and 12 achieved complete remission. However, six patients were refractory to chemotherapy and four patients with complete remission later relapsed. Eight of the 18 patients who received chemotherapy showed an aggressive disease course within a year after the diagnosis. There was a significant difference in prognosis between the group with polymorphic LPDs and the one with large cell lymphomas (p = 0.003). Although the disease profile of the 22 cases was analogous to that of immunodeficiency-associated B-cell LPDs, none of the patients showed evidence of underlying immunodeficiency-related diseases. These findings suggest that Epstein-Barr virus-associated LPD without immunodeficiency mainly occurs in elderly patients. Further investigations are needed to clarify the pathogenesis of this disease and to determine the optimal treatment strategy.

Lymex: A program in turbo-prolog for differentiating centroblastic from B-immunoblastic subtype of large cell lymphoma: U. Satyanarayana, Department Of Biophysics, All India Institute Of Medical Sciences, New Delhi, India.

Lymex: A program in turbo-prolog for differentiating centroblastic from B-immunoblastic subtype of large cell lymphoma: U. Satyanarayana, Department Of Biophysics, All India Institute Of Medical Sciences, New Delhi, India.