Single Institutional Experience on Cutaneous Manifestation of Adult T-cell Leukemia/Lymphoma - Potential Diagnostic Pitfall

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S96-S97
Author(s):  
S Bridgelall ◽  
J Michalski ◽  
X Zhang ◽  
L Sokol ◽  
N Dong ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Bone Marrow Involvement ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Cutaneous Manifestations ◽  
Skin Changes ◽  
Cutaneous Involvement ◽  
Adult T Cell Leukemia ◽  
Generalized Lymphadenopathy

Abstract Introduction/Objective Adult T-cell Leukemia/Lymphoma (ATLL) is an aggressive peripheral T-cell neoplasm caused by the human T-cell lymphotropic virus-1 (HTLV1). Approximately half of the patients diagnosed with ATLL have heterogeneous cutaneous manifestations and 1/3 of those patients have skin changes e.g. rashes, papules, and nodules at initial presentation. There is clinical and morphologic overlap between ATLL and other cutaneous T-cell neoplasms such as Cutaneous T-Cell Lymphoma/mycosis fungoides (CTCL/MF) which could pose a potential diagnostic challenge. Methods A retrospective study was conducted using PathNet system to search for HTLV1 positive ATLL patients. Clinicopathologic features of the patients with cutaneous involvement were analyzed. Results Total 31 patients with ATLL were identified. Nine patients (29%, median 54.5 years, range 47-67 years, male: female ratio 2:7) showed skin manifestations, and the cutaneous involvement with ATLL was confirmed by skin biopsy. Five (55.5%, 5/9) cases were initially misdiagnosed as CTCL/MF. Among the 5 patients, 2 presented with skin rash or diffuse erythematous patch/plaque before developing generalized lymphadenopathy or overt circulating atypical lymphocytosis; 2 developed severe pruritic rash with erythematous skin changes resembling Sezary syndrome; and 1 patient had folliculotrophic MF diagnosed 12 years before. Notably, atypical lymphocytosis (0.46- 41.19/µL) occurred in 3 of the 4 remaining cases. In addition, eight of the 9 patients displayed a variable level of CD3+/CD4+/ CD25strong+ abnormal T-cells on flow cytometry. A low-level bone marrow involvement (2-10%) was found in 8 of 9 cases. Elevated calcium levels were identified in 3 of 9 cases (33%). There were 7 of 8 patients (87.5%) who developed generalized lymphadenopathy when diagnosis of ATLL was rendered. Conclusion In patients with cutaneous manifestations, features including hypercalcemia, atypical lymphocytosis, lymphadenopathy, CD3/CD4/strong CD25 coexpression, and bone marrow involvement should prompt a test for HTLV1. Early diagnosis of ATLL can initiate proper treatment and improve patient clinical outcomes.

Clinical Characteristics and Outcomes of 102 Patients with Adult T-Cell Leukemia/Lymphoma in Peru.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 5001-5001
Author(s):  
Brady Beltran ◽  
Domingo Morales ◽  
Pilar Quinones ◽  
Jorge Castillo
Keyword(s):  
Bone Marrow ◽  
Multivariate Analysis ◽  
Prognostic Factors ◽  
T Cell ◽  
Performance Status ◽  
Bone Marrow Involvement ◽  
Type I ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Adult T Cell Leukemia

Abstract Abstract 5001 Background Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease associated with the human T-lymphotropic virus type-I (HTLV-I). ATLL has heterogeneous clinical presentations and outcomes. Shimoyama's ATLL classification includes acute, lymphomatous, chronic and smoldering subtypes. The objective of this study is to define prognostic factors for survival in patients with ATLL. Methods 102 ATLL cases were collected in our center between January 1997 and September 2008. A diagnosis of ATLL was made according to the following criteria: a clinical history consistent with ATLL, a positive HTLV-1 antibody by ELISA and Western Blot or evidence of HTLV-1 proviral integration by PCR, and histological findings compatible with ATLL. Descriptive statistics were used to assess categorical and continuous variables. Survival curves for OS were estimated using the Kaplan-Meier method and compared using the log-rank test. For the multivariate analysis, the Cox Proportional-hazard regression test was used. Results The median age at diagnosis was 60.5 years (range 23–92 years), with a female-to-male ratio of 1.2:1. Forty nine cases (48%) had a performance status ≥ 2. Clinical types were acute (n=45), lymphomatous (n=43), smoldering (n=3), cutaneous (n=10) and chronic (n=1). Median hemoglobin was 12.0 g/dl (range 5.2–17.4 g/dl), median albumin was 3.3 g/dl (range 1.8–4.6 g/dl), median beta-2 microglobulin was 4.4 g/dl (range 1.1–16.9 g/dl), and LDH was 808 UI/ml (range 298–13000 IU/ml). Twenty nine patients with acute ATLL were treated with chemotherapy obtaining an overall response rate (ORR) of 14% (4/29), 3 complete responses (CR) and 1 partial response (PR), while in 38 patients with lymphomatous ATLL, an ORR of 32% (12/38) was obtained, 8 CR and 4 PR. Smoldering and cutaneous ATL types received mainly topic treatments. Median overall survival (OS) was 2.4 months for the acute type, 11.4 months for the lymphomatous type, 17.2 months for the smoldering type and 39.4 months for the cutaneous type (p<0.00001 for trend). In the univariate analysis, presence of B symptoms (median OS 5.4 vs. 20.2 months; p=0.005), performance status ≥ 2 (median OS 3.5 vs. 15.6 months; p<0.005), clinical stage > 2 (median OS 5.7 vs. 39.4 months; p<0.005), high LDH levels (median OS 4.3 vs. 15.4 months; p<0.005), and bone marrow involvement (median OS 3.3 vs. 15.6 months; p<0.005) were associated with worse prognosis. In the multivariate analysis, bone marrow involvement and elevated LDH remained as adverse prognostic factors for survival. Conclusions ATLL is a heterogeneous disease with poor outcomes. Each ATLL subtype has distinct clinical features, including response rates and survival times. In this cohort of 102 cases, bone marrow involvement and elevated LDH levels were independent prognostic factors. Prospective studies are needed to further validate these observations. Disclosures No relevant conflicts of interest to declare.

Septic vasculitis induces cutaneous involvement of adult T‐cell leukemia/lymphoma

2020 ◽  
Vol 59 (8) ◽  
Author(s):  
Akiko Arimura ◽  
Kazuyasu Fujii ◽  
Youhei Uchida ◽  
Yuko Higashi ◽  
Naosuke Arima ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Cutaneous Involvement ◽  
Adult T Cell Leukemia ◽  
Septic Vasculitis

Specific cutaneous manifestations in adult T-cell leukemia/lymphoma

2008 ◽  
Vol 47 (4) ◽  
pp. 359-362 ◽  
Author(s):  
Fakhralzaman Pezeshkpoor ◽  
Mohammad Javad Yazdanpanah ◽  
Abbas Shirdel
Keyword(s):  
T Cell ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Cutaneous Manifestations ◽  
Adult T Cell Leukemia

Unrelated Bone Marrow Transplantation for Adult T Cell Leukemia/Lymphoma: A Study from the Japan Marrow Donor Program.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2059-2059
Author(s):  
Koji Kato ◽  
Yoshinobu Kanda ◽  
Tetsuya Eto ◽  
Tsuyoshi Muta ◽  
Hisashi Gondo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
T Cell ◽  
Marrow Transplantation ◽  
Disease Status ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Unrelated Bone Marrow Transplantation ◽  
Adult T Cell Leukemia ◽  
Related Donor

Abstract Adult T cell leukemia/lymphoma(ATLL) is known to be a very poor prognosis, and intensified chemotherapy has not yet improved this prognosis. Some successful cases of allogeneic hematopoietic stem cell transplantation (allo-HSCT) from HLA-matched siblings have been reported and allo-HSCT has been suggested as improving the prognosis of ATLL. However, HLA-matched related donor is seldom available and about two-thirds of siblings of patients with ATLL are HTLV-1 carriers. Although most of ATLL patients require an unrelated donor to benefit from allo-HSCT, there is little information about the outcome of unrelated bone marrow transplantation(UBMT) for ATLL. To elucidate the feasibility and efficacy of UBMT from HTLV-1 seronegative donor, we retrospectively analyzed the data of 33 patients who were ATLL and underwent UBMT through the Japan Marrow Donor Program. Median age of patients at transplantation was 51 years(range,24–59). The conditioning regimens of 27 patients had myeloablative intensity and 6 had reduced intensity. At transplantation, 12 patients were in complete remission, 3 were partial remission and 14 were no remission. A total of 14 patients died after allo-HSCT, and 9 of those 14 patients were lost within 100 days after HSCT due to treatment related adverse events. The overall survival(OS), progression-free survival(PFS), cumulative incidences of disease progression, and nonprogression mortality at 2 years after UBMT were 49.7%, 42.6%, 24.5%, and 32.9%, respectively. Multivariate analysis demonstrated that recipient age(P=.044) and disease status at transplantation(P=.059) were independent prognostic factors for OS and PFS. These results suggest that UBMT could be considered as a treatment option for ATLL patients without an HLA-matched related donor. Further study is required to determine the indication of nonmyeloablative conditioning based on patient’s age or disease status, because myeloablative conditioning had high incidence of treatment related mortality.

Adult T Cell Leukemia/Lymphoma Development in HTLV-1-Infected Humanized SCID Mice.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1425-1425
Author(s):  
Prabal Banerjee ◽  
Michael D Lairmore ◽  
Juan Carlos Ramos ◽  
William J Harrington ◽  
Mark A Beilke ◽  
...  
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Animal Model ◽  
T Cell ◽  
Scid Mice ◽  
Cd4 T Cell ◽  
T Cell Leukemia ◽  
Cell Leukemia ◽  
Hematopoietic Stem ◽  
Adult T Cell Leukemia

Abstract Abstract 1425 Poster Board I-448 Human T-lymphotropic virus type-1 (HTLV-1) is the first human retrovirus linked to cancer and is the etiologic agent of adult T-cell leukemia/lymphoma (ATLL), an aggressive CD4+ T cell malignancy. The molecular and genetic factors induced by HTLV-1 that initiate ATLL remain unclear, in part, due to the lack of an animal model which recapitulates leukemogenic events. In particular, early target cell infection and transformation events have not been identified or defined. Herein, we have created humanized NOD/SCID (HU-NOD/SCID) mice by inoculation of NOD/SCID mice with CD34+ hematopoietic progenitor and stem cells (CD34+ HP/HSCs) infected ex vivo with HTLV-1. HTLV-1-HU-NOD/SCID mice consistently developed CD4+ T cell lymphomas with characteristics similar to ATLL. Elevated proliferation of infected human stem cells (CD34+CD38−) in the bone marrow was observed in mice developing malignancies. Furthermore, examination of CD34+ HP/HSCs from HTLV-1-infected patients revealed proviral integrations suggesting a role of human bone marrow-derived stem cells in leukemogenesis. NOD/SCID mice reconstituted with CD34+ HP/HSCs transduced with a lentivirus vector (LV) expressing the HTLV-1 oncoprotein (Tax1) also developed CD4+ lymphomas. The recapitulation of a CD4+ T cell lymphoma in HTLV-1- and Tax1-HU-NOD/SCID mice suggest that hematopoietic stem cells serve as a viral reservoir in vivo and provide a cellular target for cell transformation in humans. This animal model of HTLV-1 induced ATLL will provide an important tool for the identification of molecular and cellular events that control the initiation and progression of the lymphoma and potential therapeutic targets to block tumor development. Disclosures: No relevant conflicts of interest to declare.

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