A patient with mycosis fungoides (MF), Kaposi\u27s sarcoma, T-cell B-cell rich lymphoma and T-cell lymphoma with angioimmunoblastic features is described. The appearance of multiple malignancies in this patient may have been caused by previous exposure to radiation in the Chernobyl accident and/or systemic chemotherapy for the initial T-cell B-cell rich lymphoma which he underwent.</p

Andrea Gat

Eli Sprecher

Ilan Goldberg

Liat Samuelov

Reuven Bergman

English

HRČAK - Portal of Croatian Scientific and Professional Journals

ACTA DERMATOVENEROLOGICA CROATICAActa Dermatovenerol Croat                                 2016;24(3):218-220                                      CASE REPORTMycosis Fungoides Associated with Kaposi’s Sarcoma, T-cell Rich B-cell Lymphoma, and T-cell Lymphoma with Angioimmunoblastic FeaturesLiat Samuelov1, Andrea Gat2, Reuven Bergman3, Eli Sprecher1,  Ilan Goldberg11Department of Dermatology, and 2Department of Pathology, Tel-Aviv Sourasky Medi-cal Center affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel; 3Department of Dermatology, Rambam Medical Center, Haifa, IsraelCorresponding author:Ilan Goldberg, MDDepartment of Dermatology Tel Aviv Sourasky Medical Center6, Weizmann StreetTel Aviv 64239Israelilangoldberg1@gmail.comReceived: November 2, 2015Accepted: July 15, 2016ABSTRACT A patient with mycosis fungoides (MF), Kaposi’s sarcoma, T-cell rich B-cell lymphoma, and T-cell lymphoma with angioimmunoblastic features is de-scribed. The appearance of multiple malignancies in this patient may have been caused by previous exposure to radiation in the Chernobyl accident and/or sys-temic chemotherapy for the initial T-cell rich B-cell lymphoma which he under-went.KEY WORDS: mycosis fungoides; Kaposi’s sarcoma; cutaneous lymphomaINTRODUCTIONMycosis fungoides (MF) is the most common type of primary cutaneous T-cell lymphoma. The disease is initially characterized by slightly scaly erythematous patches or plaques, which later may evolve into tu-mors and/or involve the entire surface of the body, finally culminating in blood and visceral dissemina-tion (1). Many clinicopathological variants of MF have been described in the literature (2). Atypical presen-tations often cause a delay in the diagnosis because these forms tend to imitate other inflammatory con-ditions.In the present case report, we describe an unusual type of MF associated with other hematological ma-lignancies in the same patient.CASE REPORTA 55-year-old man, born in Ukraine and a survi-vor of the Chernobyl disaster in 1986, was hospital-ized to investigate a disseminated skin eruption. His past medical history included ischemic heart disease, hyperlipidemia, hypothyroidism, diabetes mellitus, hypertension, and rheumatoid arthritis.  Two years prior to his admission he underwent treatment with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovine, and prednisolone) for a systemic T-cell rich B-cell lymphoma, leading to a complete remission. In addition, Kaposi’s sarcoma (KS) of the lower extremi-ties with diffuse lymph node involvement had been diagnosed one year prior to his hospitalization and was treated with vincristine and radiation.218219ACTA DERMATOVENEROLOGICA CROATICAThe patient presented with a mildly pruritic skin eruption associated with bilateral inguinal lymph node enlargement. He was previously treated with UVA1 phototherapy at another medical center where a skin biopsy had been interpreted as suggestive of morphea.The clinical examination revealed a generalized symmetric rash over the trunk, abdomen, thighs, and buttocks, composed of brown, poorly demarcated, firm, and non-tender plaques, which were considered to be morphea (Figure 1). In addition, Kaposi’s sarco-ma nodules were observed over the lower extremities (Figure 2), and bilateral inguinal lymph node enlarge-ment was detected.The routine blood tests were normal, except el-evated serum levels of C-reactive protein, lactic dehy-drogenase (LDH) and β2 microglobulin. The skin biopsy obtained from one of the plaques revealed a subepidermal, perivascular, and intersti-tial dense infiltrate of small to medium pleomorphic lymphocytes in the upper dermis. In addition, there was exocytosis of lymphocytes into the epidermis as simple units, pairs, and triplets without parakeratosis and with only slight spongiosis (Figure 3). Immuno-histochemical staining of the skin biopsies revealed a predominance of CD4+ CD3+ cells with a small num-ber of CD8+ cells. CD30+ cells were not identified. The histopathologic findings were compatible with MF. Inguinal lymph node biopsy showed complete ef-facement of the lymph node architecture due to dif-fuse infiltration of small to medium sized lymphocytes characterized by a pale cytoplasm. There was also marked proliferation of arborizing vessels (Figure 4). Immunohistochemical studies found positive stain-ing for CD3, CD4, CD5, CD10, and bcl6 of the neoplas-tic cells. Positive staining for CD21 highlighted an ex-panded dendritic framework. Stains for CD31, CD34, and factor VIII highlighted the expanded vascular proliferation, and 80% of the cells also stained posi-tive for Ki67. Staining for HHV8 (LANA) was negative.There was no evidence of Sézary cells in the blood smear. Polymerase chain reaction (PCR) analysis of DNA extracted from a lesional skin biopsy showed monoclonality of the T-cell infiltrate. Involvement of the bone marrow was ruled out by biopsy. Positron emission tomography-CT (PET-CT) demonstrated increased fluorinated deoxy glucose (FDG) uptake in the axillary, retroperitoneal, and inguinal lymph nodes.The clinical, histopathologic and immunohisto-chemical findings were compatible with a diagnosis of morphea-like mycosis fungoides (MF) in the skin and peripheral T-cell lymphoma with angioimmuno-blastic features in the lymph nodes.The patient was treated with several courses of chemotherapy, including fludarabine, cytoxan, and Figure 1. Brown, poorly demarcated, firm, non-tender plaques over the lower back.Figure 2. Pinkish nodules of Kaposi’s sarcoma over the lower extremities.Figure 3. A skin biopsy from a lesion on the trunk showing perivascular and interstitial pleomorphic lymphocytic infil-trate in the papillary dermis with focal exocytosis into the epidermis (hematoxylin-eosin ×200). Samuelov et al. Acta Dermatovenerol CroatMycosis fungoides – case report	   2016;24(3):218-220220 ACTA DERMATOVENEROLOGICA CROATICAmethotrexate, with only partial and transient re-sponse; consequently he underwent successful allo-geneic bone marrow transplantation with resolution of the skin and lymph node involvement over a two-year follow-up period. DISCUSSIONWe described three major hematological malig-nancies in a single patient: systemic T-cell rich B-cell lymphoma, MF, and T-cell lymphoma with angioim-munoblastic features. Additionally, the patient had KS. Several etiologic factors may underlie the unusual coexistence of these multiple and infrequent ma-lignancies in a relatively young individual: previous exposure to massive amounts of ionizing radiation (the patient is a survivor of the Chernobyl disaster), chemotherapy in the past due to T-cell rich B-cell lym-phoma, and the fact he was affected by an autoim-mune disease (rheumatoid arthritis).The appearance of lymphoid infiltrate in the skin lesion raised the question whether the cutaneous lesions were an expression of skin involvement by peripheral T-cell lymphoma with angioimmunoblas-tic features or MF. Cutaneous involvement of the former may be present in 50% of cases, most often as macules and papules over the trunk and extremi-ties. According to the literature, there are 4 histologic patterns of skin involvement by peripheral T cell lym-phoma with angioimmunoblastic features consisting of (a) a superficial perivascular infiltrate with or with-out atypical lymphocytes; (b) a dense superficial and deep perivascular infiltrate of atypical lymphocytes; (c) a leukocytoclastic vasculitis-like infiltrate; and (c) necrotizing granulomas (3). None of those histologic patterns were recognizable in the skin of our patient, indicating there was no secondary involvement of the skin with peripheral T-cell lymphoma with angio-immunoblastic features. In addition, the fact that the lymph node enlargement occurred prior to appear-ance of the skin lesions and the absence of histologic findings compatible with MF in the lymph nodes ruled out the possibility of lymph node involvement with MF and led to the diagnosis of two concomitant malignancies of different origin. Unfortunately, no material from the lymph node biopsy was available to molecularly compare the clones detected in the skin and in the nodal tissues. MF typically presents with slightly scaly erythem-atous or light brown macular patches, which later evolve into plaques or tumors. Several clinicopatho-logic variants have been described in the literature over the past several decades. These variants may complicate the diagnosis of the disease because the classical features of the disease may be absent or masked (4). To our knowledge, only one study pre-viously described a morpheiform type of MF resem-bling our case (5). CONCLUSIONWe have described a patient with MF presenting with morphea-like clinical characteristics associated with two other hematological malignancies and KS which may have been caused by ionizing radiation and/or previous chemotherapy. References1. Su LD, Kim YH, LeBoit PE, Swetter SM, Kohler S. Interstitial mycosis fungoides, a variant of my-cosis fungoides resembling granuloma annu-lare and inflammatory morphea. J Cutan Pathol 2002;29:135-141. 2. Martorell-Calatayud A, Botella-Estrada R, San-martín-Jimenez O, Requena C, Guillén-Barona C, Sangüeza OP. Papular mycosis fungoides: two new cases of a recently described clinicopatholo-gical variant of early mycosis fungoides. J Cutan Pathol 2010;37:330-5.3. Jayaraman AG, Cassarino D, Advani R, Kim YH, Tsai E, Kohler S. Cutaneous involvement by angioim-munoblastic T-cell lymphoma: a unique histologic presentation, mimicking an infectious etiology. J Cutan Pathol 2006;33:6-11.4. Kodama K, Fink-Puches R, Massone C, Kerl H, Cer-roni L. Papular mycosis fungoides: a new clinical variat of early mycosis fungoides. J Am Acad Der-matol 2006;55:177-80.5. Shaikh ZI, Rahman SB. Clinicopathological spectrum of mycosis fungoides type cutaneous T-cell lymp-homa. J Coll Physicians Surg Pak 2006;16:171-4.Figure 4. Inguinal lymph node biopsy showing small to medium sized lymphoid cells and marked proliferation of arborizing vessels (hematoxylin-eosin ×200).Samuelov et al. Acta Dermatovenerol CroatMycosis fungoides – case report	   2016;24(3):218-220

Mycosis Fungoides Associated with Kaposi\u27s Sarcoma, T-cell Rich B-cell Lymphoma, and T cell Lymphoma with Angioimmunoblastic Features

Abstract

Similar works

Full text

Available Versions

HRČAK - Portal of Croatian Scientific and Professional Journals

Hrčak - Portal of scientific journals of Croatia