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ALK-positive anaplastic large-cell lymphoma with primary bone involvement: A rare case and review of the literature

Noh BJ, Han CS, Park JS, Lee J, Kim YW, Park YK

Malays J Pathol, 2018 Aug;40(2):161-167.
PMID: 30173234

Primary bone lymphoma (PBL) is an uncommon type of extranodal lymphoma involvement. An anaplastic large-cell lymphoma (ALCL) is an extremely rare type of PBL, and it remains unclear whether ALCLs that primarily involve the bone exhibit favourable or unfavourable biological behaviour, and whether they are similar to ALCLs in general, or not. We reported a case of ALK-positive ALCL with primary bone involvement, and reviewed the clinicopathological features of 22 previously reported cases. An ALCL with primary bone involvement mostly affects younger patients with a preponderant towards the involvement of axial-bone. The prognosis of an ALCL that primarily involves bone is unfavourable, compared with PBL generally. The ALK-positive ALCLs in PBLs had less decedents than the ALK-negative ALCLs with a statistical non-significance (p=0.198).

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic/pathology*
Fulltext Primary bone lymphoma: report of a case with multifocal skeletal involvement

Rahmat K, Wastie M, Abdullah B

Biomed Imaging Interv J, 2007 Oct;3(4):e52.
PMID: 21614300 MyJurnal DOI: 10.2349/biij.3.4.e52

Primary bone lymphoma is an uncommon tumour accounting for approximately 4-5% of extra nodal lymphoma and less than 1% of all Non-Hodgkin's lymphoma. The radiographic appearance of primary bone lymphoma is variable. As lesions frequently resemble other disease processes namely chronic osteomyelitis and metastases, further imaging evaluation and histopathological examination allow early identification for appropriate treatment. The authors present a case of anaplastic large cell lymphoma of bone presenting with multifocal osseus involvement.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic
Cytology of Ki-1-positive anaplastic large cell lymphoma. A report of two cases

Jayaram G, Abdul Rahman N

Acta Cytol., 1997 Jul-Aug;41(4 Suppl):1253-60.
PMID: 9990253

Ki-1-positive anaplastic large cell lymphoma (Ki-1 ALCL), one of the more recently described pleomorphic types of lymphoma, affects mostly children and adolescents and is sometimes mistaken for carcinoma or sarcoma.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic/diagnosis*; Lymphoma, Large-Cell, Anaplastic/pathology*
Fulltext Primary oesophageal Ki (CD30)-positive ALK+ anaplastic large cell lymphoma of T-cell phenotype

Yaakup H, Sagap I, Fadilah SA

Singapore Med J, 2008 Oct;49(10):e289-92.
PMID: 18946602

Primary oesophageal lymphoma is a very rare entity, with fewer than 30 reported cases worldwide. It represents an important cause of dysphagia. Most of the oesophageal lymphomas are diffuse large B-cell type, with only one reported case of anaplastic large cell lymphoma (ALCL) of T-cell phenotype. Primary oesophageal lymphomas that are not associated with an immunocompromised state tend to affect elderly patients. We describe the first case of primary oesophageal Ki (CD30)-positive ALK+ALCL of T-cell phenotype in a 34-year-old immunocompetent woman, who presented with a two-year history of dysphagia. She was treated with chemotherapy and endoscopic oesophageal dilations and stenting, resulting in complete remission of the lymphoma and resolution of the dysphagia. She then underwent autologous peripheral blood haematopoietic stem cell transplantation and remained disease-free two years after the diagnosis.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic/diagnosis; Lymphoma, Large-Cell, Anaplastic/metabolism*; Lymphoma, Large-Cell, Anaplastic/pathology*
Fulltext Dendritic cell distribution in lymphomas

Hussin HN, Zulkifli FN, Phang KS, Cheong SK

Malays J Pathol, 2009 Dec;31(2):105-12.
PMID: 20514853 MyJurnal

Dendritic cells (DC) are specialized antigen presenting cells (APC) that have important roles in host defenses and in generating anti-tumour immune response. Altered frequency and maturation of DC have been reported in malignant tumours. We studied the distribution and maturation status of DC by immunohistochemistry, on the formalin-fixed, paraffin-embedded lymph node tissues of 32 histologically diagnosed lymphomas and 40 inflammatory conditions that were retrieved from the Department of Pathology, UKM Medical Centre, Kuala Lumpur. Our study showed a significant reduction in the total DC counts in the lymphoma tissues compared to the inflammatory conditions. The mature and immature DC counts were both significantly reduced (p = 0.008 and 0.001 respectively), although a greater reduction was observed in mature DC numbers. We also observed compartmentalization of DC where the immature DC were seen within the tumour tissues and the mature DC were more in peri-tumoural areas. Our findings were similar to other reports, suggesting that reduced numbers of DC appears to be a factor contributing to lack of tumour surveillance in these cases.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic/immunology; Lymphoma, Large-Cell, Anaplastic/metabolism; Lymphoma, Large-Cell, Anaplastic/pathology*
Fulltext Increased Expression of Phosphorylated FADD in Anaplastic Large Cell and Other T-Cell Lymphomas

Patel S, Murphy D, Haralambieva E, Abdulla ZA, Wong KK, Chen H, et al.

Biomark Insights, 2014;9:77-84.
PMID: 25232277 DOI: 10.4137/BMI.S16553

FAS-associated protein with death domain (FADD) is a major adaptor protein involved in extrinsic apoptosis, embryogenesis, and lymphocyte homeostasis. Although abnormalities of the FADD/death receptor apoptotic pathways have been established in tumorigenesis, fewer studies have analyzed the expression and role of phosphorylated FADD (pFADD). Our identification of FADD as a lymphoma-associated autoantigen in T-cell lymphoma patients raises the possibility that pFADD, with its correlation with cell cycle, may possess role(s) in human T-cell lymphoma development. This immunohistochemical study investigated pFADD protein expression in a range of normal tissues and lymphomas, particularly T-cell lymphomas that require improved therapies. Whereas pFADD was expressed only in scattered normal T cells, it was detected at high levels in T-cell lymphomas (eg, 84% anaplastic large cell lymphoma and 65% peripheral T cell lymphomas, not otherwise specified). The increased expression of pFADD supports further study of its clinical relevance and role in lymphomagenesis, highlighting phosphorylation of FADD as a potential therapeutic target.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic
ALK-positive anaplastic large cell lymphoma with a monomorphic small-cell pattern masquerading as inflammatory gastric lesions

Lee JS, Choi SJ, Kim L, Park IS, Han JY, Kim JM, et al.

Malays J Pathol, 2019 Aug;41(2):213-222.
PMID: 31427559

INTRODUCTION: Anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL) with a non-common pattern can be diagnostic challenging. Pathologists can be unavoidably and unintentionally blind to non-descript tumor cells in a lymphohistiocytic- (LH) or small-cell (SC)-pattern. We report a case of primary systemic ALK+ ALCL with a SC pattern that presented as secondary gastric lesions with a mixed LH and SC pattern that was masqueraded as inflammatory lesions.
CASE REPORT: A 34-year-old woman with intractable epigastric pain was referred to have repeated endoscopy with biopsy. She was found to multiple gastric erosions and nodules that were diagnosed as inflammatory lesions both endoscopically and histologically. Meanwhile, she developed an acute onset of severe back pain associated with a pathologic compression fracture in the T3 thoracic vertebral body. Imaging studies disclosed a disseminated systemic disease involving abdominopelvic lymph nodes and cervical and thoracic vertebral bodies. The needle biopsy of the pelvic lymph node disclosed diffuse proliferation of monomorphic small round cells that were diffusely positive for CD30 and ALK. A diagnosis of ALK+ ALCL with a monomorphic SC pattern was rendered.
DISCUSSION: A retrospective review of the gastric biopsies with the aid of immunohistochemistry enabled us to recognise the presence of lymphomatous infiltrates with a mixed LH and SC pattern in every piece of gastric biopsies that were repeatedly misdiagnosed as inflammatory lesions. This case illustrates a significant diagnostic pitfall of the LH- and SC-patterns in ALK+ ALCL, in which the tumour cells featuring lymphoid, plasmacytoid or histiocytoid appearance can be masqueraded as inflammatory cells.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic
Fulltext Anaplastic Large Cell Lymphoma Presenting as a Soft Tissue Mass – A Case Report

Siti-Aishah, M.A., Salwati, S., Idrus, M., Rahimah, R., Salmi, A., Leong, C.F., et al.

Medicine & Health, 2008;3(1):69-74.
MyJurnal

Anaplastic large cell lymphoma (ALCL) is a rare tumour, accounting for approximately 3% of adult non-Hodgkin lymphomas.1 Primary systemic ALCL frequently involves both lymph nodes and extranodal sites. A 44-year-old woman presented with a firm, mobile mass in the left iliac fossa region. Ultrasound findings showed a well defined inhomogenous soft tissue mass, measuring 4x4x2.6cm in the deep subcutaneous region. Histopathological examination revealed that the mass was infiltrated by large lymphoid cells with marked nuclear atypia including kidney-shaped nuclei. These neoplastic cells expressed anaplastic lymphoma kinase (ALK) (both nuclear & cytoplasmic staining), CD30 and EMA but not for T-cell (CD45RO and CD3), and B-cell (CD20 & CD79α) markers. Fluorescence in situ hybridization (FISH) analysis showed a t(2;5)(p23;q35) chromosomal translocation. Subsequently the patient developed shortness of the breath and a thoracic computed tomography (CT) scan showed a mass encasing the right upper lobe bronchus. She also had bilateral axillary lymph nodes, measuring 1 cm in diameter (biopsy was not done). The mediastinum and endobronchial region did not show any abnormalities. She received 6 cycles of CHOP chemotherapy and remained disease free 2 years after diagnosis. ALCL, rarely present as a soft tissue tumour and this disease should be included as a differential diagnosis of any soft tissue mass.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic
Fulltext Leukemic phase of ALK-negative anaplastic large cell lymphoma in a patient who is on androgenic steroids: A case report

Kasinathan G

Ann Med Surg (Lond), 2020 Jan;49:1-4.
PMID: 31871676 DOI: 10.1016/j.amsu.2019.11.007

ALK-negative anaplastic large cell lymphoma (ALCL) is a peripheral T-cell lymphoma that usually involves lymph nodes or extranodal sites. Leukemic phase of ALK-negative ALCL is exceedingly rare and often carries a poor prognosis. Androgenic steroids have gained popularity among the young, and at higher doses, it can result in immune dysregulation and may be potentially carcinogenic. Case presentation: A 30-year-old gentleman of Malay ethnicity presented to the hematology department with night fevers, loss of weight and bony pain for the past 6 weeks. He is a gymnasium instructor with a history of chronic usage of intramuscular testosterone enanthate. Physical examination revealed ecchymosis over the left elbow and hepatomegaly. A complete blood count depicted anemia, thrombocytopenia and leucocytosis. An 18-Fluorodeoxyglucose positron emission tomography (18-FDG PET/CT) imaging showed a hypermetabolic anterior mediastinal mass of 6.8 × 7.0 × 6.5 cm with diffuse hypermetabolism in the liver, spleen and axial skeleton. The bone marrow trephine and mediastinal tissue histology were consistent with leukemic ALK-negative ALCL. He was treated with CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisolone) induction chemotherapy in which he required intensive antibiotic and blood support. He progressed with worsening B symptoms and new diffuse lymphadenopathies suggesting rapid dissemination of the disease. He subsequently succumbed to multiorgan failure with disseminated intravascular coagulopathy at the intensive care unit. Conclusion: Leukemic phase ALK-negative ALCL often carries a complex karyotype and requires early intensive polychemotherapy. Use of anabolic steroids depletes the ability of defending lymphocytes to remove tumour producing cells.

Matched MeSH terms: Lymphoma, Large-Cell, Anaplastic