Mantle cell lymphoma concurrent with T-large granular lymphocytic leukemia: report of a case and review of literature

Mantle cell lymphoma is one of the B-cell lymphomas. The concurrent presentation of mantle cell lymphoma with large granular lymphocytic leukemia simultaneously has never been reported. In this case we present an old man with concomitant mantle cell lymphoma and large granular lymphocytic leukemia diagnosed by the morphology of the bone marrow aspiration, immunophenotyping of the peripheral blood by flow cytometry detecting the increased CD3+CD4-CD8+ cells, immunohistochemical studies of lymph node showed cyclinD1+, chromosome analysis by fluorescence in situ hybridization (FISH) showed t(11,14), positive results of IGH and TCR rearrangement studies. The patient discharged from the hospital voluntarily and lost the follow-up. A brief discussion is also presented.     

血管内淋巴瘤

目的：探讨血管内淋巴瘤的临床病理特征。方法：对1例血管内淋巴瘤进行免疫表型分析及原位杂交检测与EB病毒的关系,并文献复习,结果：女性,48岁,不明原因发热伴体重下降3个月,CT扫描示子宫肿块而行子宫加双侧附件切除,病理学检查发现瘤细胞位于血管内,伴少许管周浸润,瘤细胞免疫表型CD45（LCA）和CD20（L26）呈阳性表达,原位杂交显示E－BERs阴性,患者经CHOP联合化疗后获得完全缓解,结论：血管内淋巴瘤是一种极罕见的B细胞肿瘤,此瘤的诊断只能依赖病理学检查,治疗上应予以积极联合化疗。     

De novo hairy cell leukemia with a major BCR/ABL1 rearrangement: A case report with a literature review

Hairy cell leukemia (HCL) is a very rare mature B‐cell neoplasm and its simultaneous occurrence with chronic myeloid leukemia has been reported in only three cases. The pathogenesis and relationship of the two diseases are not clear. Here we report a case of HCL expressing a BCR/ABL1 clone, which showed molecular remission of the fusion clones and achieved partial remission over nine months of cladribine therapy. After a thorough analysis of previous studies and the results of this patient, we speculate that a subclone evolved to have an additional genetic BCR/ABL1 rearrangement. We also review all published literature on HCL with BCR/ABL1 rearrangement and discuss the pathophysiology of these unusual cases.     

Composite T lymphoblastic leukemia/lymphoma and diffuse large B-cell lymphoma: case report

This report concerns a unique case of a composite lymphoma composed of T-lymphoblastic leukemia/lymphoma (T-LBL) and diffuse large B-cell lymphoma (DLBCL) in a 72-year-old woman with generalized lymphadenopathy, splenomegaly and ascites. Laboratory findings showed increased lactate dehydrogenase and soluble interleukin-2 receptor. The biopsy specimen showed replacement of the normal architecture of the lymph nodes by a tumor containing a dual cell population composed of large lymphocytes and medium-sized lymphocytes. Sheets of large lymphocytes often were punctuated by clusters of medium-sized lymphocytes. Flow cytometry and immunohistochemical analysis showed a composite lymphoma with both T-LBL and DLBCL. The T-LBL expressed CD1a, CD3, CD4, CD8, and terminal deoxynucleotidyl transferase. The DLBCL expressed CD19 and CD20, CD23, bcl-2, bcl-6, MUM1 and immunoglobulin κ light chain. Polymerase chain reaction detected a monoclonal pattern of T-cell receptor γ and immunoglobulin heavy chain rearrangements in the same specimen. She received eight cycles of R-CHOP (rituximab+cyclophosphamide, doxorubicin, vincristine, prednisone) therapy and achieved complete remission. She has shown no signs of recurrence 20 months after the diagnosis. We describe here a very unusual and, to the best of our knowledge, an as yet never reported case of a primary composite lymphoma of T-LBL and DLBCL.     

ALK-negative anaplastic large cell lymphoma primarily involving the bronchus: a case report and literature review

Anaplastic large cell lymphoma (ALCL) is a mature T cell lymphoma with characteristic morphologic, immunophenotypic and cytogenetic features. Current WHO classification includes anaplastic lymphoma kinase (ALK)-positive and ALK-negative variants. ALCL rarely presents with obstructive symptoms of the main airway. In addition to reporting a HIV-associated bronchial ALK-negative ALCL in a 44 year-old female, our literature review identified eight cases of bronchial ALCL with several interesting clinicopathological features, including: 1) a female predominance (67%); 2) two thirds of patients younger than 18 years old; 3) uniformly presented with respiratory symptoms and progressed to respiratory failure; 4) the tumor involving the main airways; 5) often with localized disease at the initial presentation. This unusual presentation of ALCL may pose as a diagnostic pitfall and delay the treatment.     

淋巴瘤样肉芽肿型大B细胞淋巴瘤

目的 探讨淋巴瘤样肉芽肿型大B细胞淋巴瘤(原名淋巴瘤样肉芽肿病,lymphomatoid granulomatosis,LYG)的病理形态特征、病变性质及鉴别诊断要点。方法 对l例LYG的组织形态学、免疫组织化学、EBV原位分子杂交(EBER)结合临床特征进行了分析。结果 l例63岁男性患者,临床上表现为多系统多器官性病变,主要累及肺,表现为双肺内境界清楚的圆形结节,呈孤立性或弥漫性分布,并出现多发性皮下结节,发热、体重减轻、全身无力等症状。皮下结节活检示多个肉芽肿样结构,细胞形态多样,见组织样细胞、非典型淋巴样细胞、小淋巴细胞、浆细胞及散在多核巨细胞,可见一血管壁有淋巴样细胞浸润,未见明显中心粒细胞,可见核分裂象。肺部穿刺组织示弥漫淋巴样细胞浸润,并见灶性坏死,免疫表型示瘤细胞呈CD20 ,CD79α ,CD43 ,CD3-,GraB-,EBV散在 ,CK-,Syn-,原位杂交示EBER 。结论 本例LYG是一种罕见的淋巴瘤样肉芽肿型大B细胞性淋巴瘤,与EBV相关。临床与影像学上与Wegener肉芽肿相似。肺部出现结节状病灶时,临床上易与结核、肉芽肿病、肺癌及炎性假瘤等相混淆,病理上须与结核、非特异性肉芽肿病、结外的外周T细胞淋巴瘤等相鉴别,形态学、免疫表型结合临床特征可明确诊断。     

Anaplastic large cell lymphoma involving the urinary bladder: A case report and review of the literature

T cell‐derived malignant lymphoma is rarely detected as a bladder neoplasm. A literature review for anaplastic large cell lymphoma (ALCL) involving urinary bladder reveals only seven previously reported cases. Here, we report a case of a 59‐year‐old HIV‐negative man with ALK‐positive ALCL. He presented an unusual clinical course with initial consideration of adult onset Still's Disease (AOSD) due to his negative results searching for malignancy and infectious diseases. He rapidly developed macrophage activation (hemophagocytic) syndrome and experienced an unusual rapid disease progression and died in 39 days after onset of symptoms. Compared to previously reported cases, the current case of ALK‐1‐positive ALCL is a rare case with an unusual presentation. From this case, we learned that ALCL is one malignancy that should be considered and screened in patients with suspected AOSD. Also, T‐cell lymphoma associated hemophagocytic syndrome should be considered in a patient with sustained corticosteroid‐resistant spike fever, high serum ferritin, and rapid exacerbation of the disease course. Diagn. Cytopathol. 2015;43:60–65. © 2014 Wiley Periodicals, Inc.     

CD20dim-positive T-cell large granular lymphocytic leukemia in a patient with concurrent hairy cell leukemia and plasma cell myeloma

We report a CD20dim- positive T-cell large granular lymphocytic (T-LGL) leukemia in a patient with concurrent hairy cell leukemia and plasma cell myeloma. This patient was first diagnosed with T-LGL leukemia with dim CD20 expression, which by itself was a rare entity. He received no treatment for T-LGL leukemia. The patient later developed a hairy cell leukemia, which went into complete clinical remission after one cycle of 2-CdA. Five years later, he was diagnosed with a third malignancy, plasma cell myeloma. Complex cytogenetic aberrancies were present at the time when plasma cell myeloma was diagnosed. This is the first report, to the best of our knowledge, in the English literature with the aforementioned three distinct hematopoietic malignancies in one patient.     

肉瘤样型间变性大细胞淋巴瘤临床病理特征

目的探讨肉瘤样型间变性大细胞淋巴瘤(sALCL)临床病理特点、免疫表型及分子遗传学特征。方法对1例sALCL的临床、病理组织学、免疫表型及免疫球蛋白重链(IgH)和T细胞受体(TCR)基因克隆性重排情况进行观察并复习相关文献。结果眼观:送检淋巴结1枚,1.5cm×1.0cm×1.0cm,切面呈鱼肉状。镜检:淋巴结基本结构几乎完全被破坏,异型的梭形和上皮样细胞弥漫增生。免疫表型:瘤细胞呈CD30、ALK1、EMA、CD45RO、CD45、TIA1、granzymeB、perforin、CD68(部分)、SMA(梭形成分)阳性。基因重排:TCRβ1克隆性重排。结论sALCL属罕见恶性肿瘤,其形态不典型,易误诊为其他恶性肿瘤,免疫表型和遗传学异常有助于其诊断和鉴别诊断。     

CD56 positive diffuse large B-cell lymphoma: a case report and literature review

CD56 positive B-cell lymphoma is very rare. We experienced a case of CD56 positive diffuse large B-cell lymphoma, occurred in a young child. A 5-year-old girl complained with snoring and open mouth breathing. No any abnormality in laboratory or physical examination was present, except enlarged both tonsils. Bilateral tonsillectomy was performed. Cut sections of right tonsil showed a 2 cm size, solid mass. On microscopically, large monomorphic lymphoid cells were diffusely proliferated and showed positivity for CD20 and CD56 and negative for Epstein-Barr virus (EBV) polymerase chain reaction (PCR). Monoclonality was observed on immunoglobulin heavy chain gene rearrangement. This is a unique case with incidentally found and occurred in a young child.     

Extra-oral plasmablastic lymphoma: report of a case and review of literature

Plasmablastic lymphoma (PBL) of the oral cavity is classified as one subtype of diffuse large B-cell lymphoma that is most commonly seen in patients with human immunodeficiency virus infection. We report a rare case of PBL in the anal canal of a 33-year-old man with human immunodeficiency virus infection. The lymphoma cells were positive for CD138 and weakly positive for CD79a. In addition, these cells were also positive for CD10. The neoplastic cells were positive for Epstein-Barr virus and negative for human herpes virus 8. Review of the English medical literature revealed many more cases of extra-oral PBL. We propose that the term plasmablastic lymphoma of the oral cavity in World Health Organization classification be revised to simply plasmablastic lymphoma, which would include both oral and extra-oral PBLs, and the term to define the primary site of the lymphoma (ie, oral cavity) be dropped from the terminology used in World Health Organization classification.     

Adult T-cell leukemia/lymphoma accompanying follicular mucinosis: a case report with review of the literature

Follicular mucinosis is recognized as one of the histopathological reaction patterns characterized by the accumulation of mucin within follicular epithelium. It is induced by various causes including inflammatory diseases, and more than half of the cases are associated with malignant lymphoma, mainly mycosis fungoides. Herein, we describe the third documented case of adult T-cell leukemia/lymphoma (ATLL) accompanying follicular mucinosis. A 72-year-old Japanese male presented with persistent erythema in his arm and neck. Laboratory tests demonstrated positivity for human T-cell leukemia virus (HTLV)-1 antibodies. Histopathological study of the biopsy specimen from the neck revealed superficial perivascular, nodular, and intrafollicular lymphocytic infiltrations. These lymphocytes were small- to medium-sized and had convoluted nuclei. Mucoid material deposition was observed within the hair follicles, and it was digested by hyaluronidase. Immunohistochemically, these lymphocytes were positive for CD3, CD4, CD25, and Foxp3. Accordingly, an ultimate diagnosis of ATLL accompanying follicular mucinosis was made. The skin is the most common extralymphatic site of involvement of ATLL. The present case clearly demonstrated that albeit extremely rare, ATLL can cause follicular mucinosis. Therefore, ATLL should be included in the differential diagnostic consideration of follicular mucinosis.     

Composite primary breast diffuse large B-cell lymphoma and T lymphoblastic leukemia/lymphoma: report of a case and review of literature

We reported a rare case of composite diffuse large B-cell lymphoma and T lymphoblastic leukemia/lymphoma (T-LBL) in a 46-year-old woman with progressive enlargement of the breast lump. The patient initially sought care at a local hospital with a single left breast lump without any other physical examination findings. Histopathological analysis of which revealed a diffuse infiltration of tumor cells that were rich in cytoplasm with vesicular chromatin and prominent nucleoli. Further analysis of immunohistochemistry showed a cluster of neoplastic cells which express B-cell markers: CD19, CD20 (weak), CD79a, PAX5 and BCL-2, but negative for T-cell markers such as CD2, CD3, CD5 and CD7. PET-CT showed evidence of lymphadenopathy and splenomegaly, which may indicate lymphoma infiltration. Then a biopsy of bone marrow showed typical features of T-LBL. The aberrant terminal deoxynucleotidyl transferase (TDT) and cCD3 positive T-cell population that lack surface CD10 and CD19 were identified by flow cytometric immunophenotyping. Polymerase chain reaction analysis of the T-cell receptor gamma gene and IgH gene revealed a clonal rearrangement and confirming T-cell clonality. Fluorescence in-situ hybridization (FISH) revealed a deletion of the P53 gene in these T-neoplastic cells may indicate a bad outcome of such disease. Neither the large B-cells nor T-cells were positive for Epstein-Barr virus encoded RNA.     

Intravascular large B-cell lymphoma secondary to lymphoplasmacytic lymphoma: a case report and review of literature with clonality analysis

Intravascular large B-cell lymphoma (IVLBCL) can be a fatal malignancy mainly because of difficulty in early detection. Due to the lack of specific clinical manifestations, early detection of IVLBCL remains a challenge, especially in the presence of comorbidities. Lymphoplasmacytic lymphoma (LPL) is an indolent B-cell lymphoma accompanied by monoclonal immunoglobulin M protein in most patients, and known to be associated with high risk of secondary hematological malignancies. Here, we report a patient who developed IVLBCL during treatment for LPL that presented a diagnostic challenge. Rearrangement analysis of the immunoglobulin heavy chain revealed the different clonal origins of two lymphomas, implying a predisposition of LPL to develop unrelated secondary lymphoma. Secondary lymphoma including IVLBCL during the treatment for LPL deserves consideration in order to facilitate early diagnosis and intervention.     

Cytomorphologic features of primary anaplastic large cell lymphoma of the psoas muscle: A case report and literature review

Ki‐1 (CD30) positive anaplastic large cell lymphoma (ALCL) is an uncommon malignancy, which may present with nodal as well as extra‐nodal disease. Primary skeletal muscle Ki‐1 (CD30) positive ALCL is an even rarer event with few cases reported in the literature and only some with published cytomorphologic features. An 83‐year‐old woman underwent a fine needle aspiration (FNA) of a psoas muscle mass. Smears demonstrated a predominantly discohesive population of large pleomorphic cells. The nuclei were hypechromatic and lobulated, with often multinucleation. Nucleoli were prominent in a subset of cells. Cytoplasmic vacuolization was also present. No lymphoglandular bodies were identified. A cytodiagnosis of malignant cells favoring metastatic melanoma vs. poorly differentiated carcinoma was rendered. Morphologic and immunohistochemical features later revealed a primary psoas muscle Ki‐1 (CD30) positive ALCL with negative staining for anaplastic large cell lymphoma kinase (ALK). Cytologic features of ALCL mimic epithelial neoplasms, sarcomas, melanoma and other lymphomas. Although rare, ALCL should be a diagnostic consideration when discohesive pleomorphic malignant cells are encountered on FNA of a muscle neoplasm. Diagn. Cytopathol. 2010. © 2009 Wiley‐Liss, Inc.     

Clear cell variant of diffuse large B-cell lymphoma: a case report and review of the literature

Diffuse large B cell lymphoma (DLBCL) is a diffuse proliferation of large neoplastic B lymphoid cells with nuclear size equal to or exceeding that of normal macrophage nuclei. The DLBCL morphological variants are centroblastic, immunoblastic, T-cell- and histiocyte-rich, anaplastic, plasmablastic, anaplastic lymphoma kinase-positive, and primary mediastinal large B-cell lymphoma (PMBCL). These histopathologically-recognized morphological variants respond differently to treatment and have distinct prognoses. We report a case of a 43-year-old patient who presented pain in the lower abdomen that had begun four months prior. Ultrasound-guided biopsy revealed epithelial cell features and a partial alveolar growth pattern. We discovered large diffuse areas comprising large cells with slightly irregular nuclei and very clear cytoplasm. These features were similar to those of clear cell carcinoma in renal tissue, suggesting the possibility of an epithelial neoplasm. To test this possibility, immunohistochemistry for cluster designation markers was performed, but the diffuse areas were found to be positive only for CD45. Additional immunohistochemistry was performed, and the diffuse areas were found to be positive for CD20, CD79a, P53, and Mum-1. Based on these characteristics, a diagnosis of a clear cell variant of DLBCL was made, and the patient was treated with chemotherapy. Precise histological diagnosis is crucial for clinical management and ultimately for patient survival. There has been one additional report of a case of clear cell DLBCL, in outside the mediastinum. The features we identified can be used to define a new subtype of DLBCL. The expression of P53 and Mum-1 suggest a poor prognosis.     

Primary thyroid T-lymphoblastic lymphoma: a case report and review of the literature

Most thyroid lymphomas are B-lineage, and T-cell lymphomas are rare. Here, we report a case of primary T-cell lymphoblastic lymphoma (T-LBL) of the thyroid gland. A 15-year-old boy presented with a painless thyroid mass. Ultrasonographic examination revealed a hypoechoic thyroid nodule measuring 4.6 cm × 1.9 cm × 3.4 cm. The thyroid function and antibodies were normal. Hemithyroidectomy was performed. Intraoperative frozen section was suggestive of malignant lymphoma. Histological examination showed diffuse round to oval medium sized cells with high nuclear/cytoplasmic ratio, finely dispersed chromatin, scanty cytoplasm, and numerous mitoses. Immunohistochemical studies revealed malignant cells were positive for terminal deoxynucleotidyltransferase, CD5, CD7, CD8, CD10, CD45RO, CD99, CD79a, CD3, CD1a and Ki-67 (>40%) and negative for CD34, CD20, BCL6, CD23, BCL2, Pax5 and EBV. A diagnosis of thyroid T-LBL was made. The patient was treated by intensive chemotherapy followed by allogeneic hematopoietic stem cell transplantation and has been in event-free survival for 65 months. The patient was unique because no cases of thyroid T-LBL have been previously reported, to our knowledge. Moreover, intensive chemotherapy followed by alloHSCT might be one of the adoptive options in therapy for this aggressive disease.     

Primary cerebral anaplastic large cell lymphoma containing abundant reactive histiocytes and eosinophils. A case report and literature review

Primary cerebral anaplastic large cell lymphoma (ALCL) is very rare. We report on our experience with such a case and review the literature. A 46-year-old Taiwanese woman presented with headache, weakness of her right extremity, and limited eye movement. A solid mass (5 cm x 4 cm) at the left occipital lobe was almost completely removed. The neoplastic cells, some of which had reniform or embryo-like nuclei, were large and were admixed with abundant eosinophils, histiocytes, and some small lymphocytes. These neoplastic cells expressed CD30, CD43, granzyme B and T-cell intracellular antigen-1, but not ALK1, CD3, CD20, CD45, CD79a, cytokeratin, and EMA. They were positive for Epstein-Barr virus-encoded mRNA by in situ hybridization. Polymerase chain reaction study of formalin-fixed tissue showed a clonal gene arrangement of the T-cell receptor-gamma chain. ALCL of T-cell lineage with cytotoxic phenotype was diagnosed. The patient received cranial irradiation and has remained with no evidence of disease for 25 months of follow-up.     

Development of angioimmunoblastic T-cell lymphoma after treatment of diffuse large B-cell lymphoma: a case report and review of literature

Cases of diffuse large B-cell lymphoma (DLBCL) arising after the initial diagnosis of angioimmunoblastic T-cell lymphoma (AITL) and DLBCL synchronous with AITL have been reported. To date, there is no report on the subsequent development of AITL in patients with DLBCL. Here we presented a rare case of AITL developing six months after the initial diagnosis of DLBCL. In order to investigate the clinical and molecular features of patients with AITL and DLBCL, we also reviewed the literature on AITL patients developing DLBCL, and patients with composite AITL and DLBCL.