Primary cutaneous lymphoma in a patient with mastocytosis: Is there an association?

• DOI: 10.1016/j.jdcr.2023.03.015
• Publication Date: 2023-04-08T00:25:04Z
• Authors: Colin M. Kincaid; Celine Phong; Justin D. Arnold; Xiying Fan; Bonnie A. Lee; Natasha A. Mesink...
• Keywords: Cutaneous Mastocytosis

Mast cells, classically known as local effector cells in allergic reactions, have become recognized recent years for their pathogenic contributions to tumor promoting microenvironments.1Rabenhorst A. Schlaak M. Heukamp L.C. et al.Mast play a protumorigenic role primary cutaneous lymphoma.Blood. 2012; 120: 2042-2054https://doi.org/10.1182/blood-2012-03-415638Crossref PubMed Scopus (106) Google Scholar Patients with mastocytosis are at an increased risk myeloid malignancies, emerging evidence of co-occurrence subset lymphoproliferative disorders, lymphomas (PCL).2Günay M.B. Büyükbabani N. Yavuz A.S. Yılmaz I. Baykal C. Association systemic marginal zone lymphoma; first case.J Eur Acad Dermatol Venereol. 2022; 36: e275-e276https://doi.org/10.1111/jdv.17797Crossref (1) Because the presentation PCL lesions can be asymptomatic and subtle, we aim alert dermatologists clinical clues patients longstanding mastocytosis. A 35-year-old male 7-year history biopsy-proven mastocytosis, well controlled antihistamines dietary avoidance, presented clinic annual surveillance. Clinical examination revealed numerous brown macules on his trunk, consistent urticaria pigmentosa, unchanged from prior examinations. Involving right vertex scalp, he had new 10 mm smooth, dome shaped, red-to-violaceous nodule rare hairs (Fig 1, B). He noticed slowly enlarging 4 months thought it was cyst. Lymphadenopathy not detected patient denied any constitutional symptoms. His laboratory workup, including complete blood count, serum tryptase, 24-hour urinary N-methylhistamine, abdominal ultrasound were unremarkable. declined bone marrow biopsy which is part workup (SM) per World Health Organization (WHO) guidelines.3Pardanani Systemic adults: 2021 update diagnosis, stratification management.Am J Hematol. 2021; 96: 508-525https://doi.org/10.1002/ajh.26118Crossref (79) The instructed return if lesion did resolve within 6 8 weeks. On return, slightly more elevated. Biopsy nodular diffuse infiltrate composed enlarged lymphocytes abundant cytoplasm, aggregates periphery C). Immunohistochemical studies notable diffusely positive CD20 (cluster differentiation 20) BCL6 (B-cell lymphoma 6) staining D), germinal center B cells. BCL2 significant CD21 expanded network follicular dendritic CD117 stain mast negative. Gene rearrangement confirmed presence monoclonal cell population. diagnosis B-cell lymphoma, follicle type, made. Treatment options, triamcinolone injections, excision, radiation considered. elected series monthly intralesional injections (0.5 mL 10-20 mg/mL), resulted improvement—but relapses—before eventually achieving control. Mastocytosis, characterized by proliferation accumulation skin or other organs, associated hematologic neoplasm (AHN) up 40% all SM cases.3Pardanani classification "SM-AHN" thus one main subtypes defined WHO. Approximately 89% SM-AHNs neoplasms that often attributed activating c-KIT mutations shared clonal progenitor.3Pardanani Interestingly, 10% SM-AHN disorders. But given distinct origins lymphocytes, this association remains unclear.2Günay diagnostic finding young surprising may consider completely coincidental. However, interact through production soluble cell-membrane factors4Palma A.M. Hanes M.R. Marshall J.S. modulation responses: under-appreciated partnership host defence.Front Immunol. 12718499https://doi.org/10.3389/fimmu.2021.718499Crossref (11) contribute pro-tumorigenic microenvironments.5Komi D.E.A. Redegeld F.A. Role shaping microenvironment.Clin Rev Allergy 2020; 58: 313-325https://doi.org/10.1007/s12016-019-08753-wCrossref (144) In malignant neoplastic processes, derived cytokines, matrix metalloproteinases, vascular endothelial growth factors strongly promote proliferation, tissue remodeling, angiogenesis, respectively.5Komi Historically, disorders reported traditionally been lymphomas.2Günay question case raises possibility whether skin-residing also similar fashion certain neoplasms. strong support this, even numbers, both B- T-cell types importance supporting has demonstrated in-vivo experiments animal models.1Rabenhorst Subsequent experimental evidence, there 3 additional cases reporting type (Table I).2Günay Scholar,6Meyer K.M. Landthaler Hafner Geissinger E. lymphoma.Br Dermatol. 2013; 169: 1165-1167https://doi.org/10.1111/bjd.12471Crossref (3) Scholar,7Lee H.Y. Lee Koo D.W. report mastocytosis.SAGE Open Med Case Rep. 92050313X211042527https://doi.org/10.1177/2050313X211042527Crossref conjunction present case, contemplate these represent previously underrecognized connection. While PCLs heterogenous group varied presentation, few described appear some common features. They onset, nodules plaques, very monomorphic, underlying development tumors affected (age range, 31-53 years) occurred 7 after initial diagnosis. It too precocious ask exercise heightened index suspicion when evaluating novel patients. would like reader growing body promotors microenvironments.Table ISummary mastocytosisAuthorAge (y)SexMastocytosis presentationTime diagnosisPCL presentationPCL diagnosisMeyer al 20136Meyer Scholar41MBrown-reddish trunk3 yMultiple bluish purple, 2-3 cm plaques chestFollicular lymphomaLee 20217Lee Scholar53MMultiple dusky red trunk7 trunk (overlap presentation)Marginal lymphomaGuney 20222Günay Scholar31FBrownish, monomorphic extremities3 yFour reddish, 1-2 upper armMarginal lymphomaPresent Case35MBrown ySingle scalpFollicular lymphomaPCL, Primary lymphoma. table tab PCL, None disclosed.