scholarly journals CD8+ mycosis fungoides: A wolf in sheep’s clothing?

2022 ◽  
Vol 13 (1) ◽  
pp. 118-119
Author(s):  
Asmae Abdelmouttalib ◽  
Sanae Sialiti ◽  
Soumaya Hamich ◽  
Kawtar Znati ◽  
Mariame Meziane ◽  
...  

Sir, CD8+ mycosis fungoides (MF) is a rare form of MF with an indolent course. Herein, we report a rare case of CD8+ fungoid mycosis preceded by lymphomatoid papulosis type D with an aggressive course. A 36-year-old female presented with several papular lesions on the trunk and extremities with a relapsing–remitting course. Histopathology and an immunohistochemical study confirmed CD8-positive lymphomatoid papulosis type D and methotrexate at 25 mg/week was initiated. After a temporary clinical improvement, the lesions worsened, became infiltrated, and grouped as vaguely annular and angular patches with serpiginous borders (Fig. 1). A second scalp biopsy was performed and a diagnosis of CD8+ MF was established. An extension workup was normal, and MF was classified as stage IB. PUVA therapy was started with acitretin at 25 mg/day. After four weeks from the beginning of treatment, the patches completely disappeared but with the concomitant appearance of four subcutaneous tumors. The evolution was spectacular in fifteen days, with the tumors rapidly increasing in size, becoming ulcerative and necrotic, and one being localized in the left cervical area compressing the upper respiratory tract (Fig. 2). A subsequent biopsy revealed massive epidermal and dermal large cell infiltration (Fig. 3a); the tumor cells were positive for CD3, CD8, and CD7 (Fig. 3b) and negative for CD4, CD5, CD3, CD2, and CD30. Antigen Ki-67 was expressed by more than 80% of T-cells (Fig. 3c). A cerebral and thoraco-abdominal CT scan revealed multiple axillary lymph nodes with hypermetabolism on a PET scan. An osteomedullar biopsy was normal, and lactate dehydrogenase (LDH) was increased to 358 U/L. Chemotherapy was performed, but the patient died after two cycles of CHOEP. In contrast to classical CD4+ mycosis fungoides, CD8+ MF is a rare cytotoxic phenotype constituting about 5% of all cases of MF [1]. It belongs to the first group of primary cytotoxic cutaneous lymphomas (PCCL) with a good prognosis, an indolent course, and a slow progression of the lesions over several years [2]. However, rare cases with a more aggressive course have been reported in the literature [3]. The main differential diagnosis of aggressive CD8+ MF is an aggressive epidermotropic cutaneous CD8+ lymphoma that is a rare cutaneous lymphoma with a poor prognosis due to rapid extracutaneous dissemination [4]. The prognosis of the CD8+ subtype of mycosis fungoides (MF) is controversial. More studies are necessary to clarify this subject.

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1487-1487
Author(s):  
Iris Wieser ◽  
Chee Won Oh ◽  
Rakhshandra Talpur ◽  
Madeleine Duvic

Abstract Introduction: Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology. Objective: The aim of the study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center. Methods: This was a retrospective study of patients with LyP seen between 1999 and 2015. Patient charts, clinical and histopathological data were evaluated, searching the MD Anderson Cancer Center cutaneous T-cell lymphoma database. A total of 237 patients with the diagnosis of LyP were identified, with 180 patients meeting the inclusion criteria of both clinical diagnosis of LyP and confirmatory biopsy. Results: A total of56.6% of patients were male and 43.3% were female. The majority of patients were Caucasians (77.7%, n=139). The mean age at diagnosis was 52.7±17.2 years. The mean time from symptom onset to a biopsy proven diagnosis of LyP was 45.0±75.4 months. The majority of patients (51.6%, n=83) had less than twelve lesions at the worst time point. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6% and mixed subtype in 4.4% of the patients. Associated lymphomas (n=114) were observed in 93 patients with mycosis fungoides (61.4%, n=70) and anaplastic large cell lymphoma (26.3%, n=30) being the most common forms (Table). Men were 2.85 times (95%CI: 1.55-5.24) more likely to develop a secondary lymphoma than women. Other risk factors for development of lymphoma included histologic subtypes B and C and older age. Number of lesions, symptom severity and ethnicity were not associated with lymphoma development. Patients with type D (CD8+) were less likely to develop lymphomas. Despite the high number of detected secondary malignancies, the prognosis of LyP is excellent. Only eight out of 180 evaluated patients died of their disease. A broad variety of treatment options are available for symptomatic relief only, since no treatment modality was found to prevent progression to lymphoma. Conclusion: We could demonstrate that LyP is associated with other cutaneous and extra-cutaneous malignancies, the most common being MF and ALCL. To date the risk of developing associated lymphomas cannot be altered by medication. Long term follow-up exams and proper patient counseling are required to ensure early detection of evolving secondary malignancies. Table 1. A total of 114 hematologic malignancies were observed in 93 patients with lymphomatoid papulosis diagnosed before, concomitant and after diagnosis of lymphomatoid papulosis Hematologic malignancy Before (n=39) Concomitant (n=28) After (n=47) All (n=114) %(n) %(n) %(n) %(n) MF (all stages) 17.5% (20) 17.5%(20) 26.3%(30) 61.4% (70) MFIA 9.6% (11) 7.0% (8) 20.2%(23) 36.8%(42) MFIB 4.4% (5) 4.4% (5) 2.6%(3) 11.4%(13) MFIIA 1.0% (1) - - 1.0%(1) MFIIB 1.0% (1) 1.0% (1) 1.0%(1) 2.6%(3) MFIVA - 1.0% (1) - 1.0% (1) FMFIA - 1.8% (2) 1.0%(1) 2.6%(3) FMFIB 1.8%(2) 1.0% (1) - 2.6%(3) FMFIIB - 1.0% (1) - 1.0% (1) FMFIVA - - 1.0%(1) 1.0% (1) Hypopigmented MF - - 1.0%(1) 1.0% (1) Granulomatous MF - 1.0% (1) - 1.0% (1) ALCL 12.3%(14) 7.0% (8) 7,0%(8) 26.3% (30) HD 1.0%(1) - 2.6%(3) 3.5%(4) CLL 2.6%(3) - 1.0%(1) 3.5%(4) AML - - 1.0%(1) 1.0% (1) B-cell lymphoma - - 1.8%(2) 1.8% (2) LGL 1.0%(1) - 1.0%(1) 1.8% (2) Multiple Myeloma - - 1.0%(1) 1.0% (1) Abbreviations: ALCL, anaplastic large cell lymphoma; AML, acute myeloid leukemia; CLL, chronic lymphatic leukemia; FMF, folliculotropic mycosis fungoides; HD, Hodgkin disease; LGL, T-cell large granular lymphocytic leukemia; MF, mycosis fungoides; n, number of lymphoma cases. Disclosures Duvic: Therakos: Research Funding.


2018 ◽  
Vol 58 (7) ◽  
pp. 800-805 ◽  
Author(s):  
Olivia C. Simo ◽  
Simon J. Warren ◽  
Lawrence Mark ◽  
Kristin Hoffmann ◽  
Ahmed K. Alomari

2018 ◽  
Vol 28 (2) ◽  
pp. 267-268
Author(s):  
Junko Hori-Kosogabe ◽  
Toshihisa Hamada ◽  
Shin Morizane ◽  
Yoji Hirai ◽  
Tomoko Miyake ◽  
...  

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 464-472 ◽  
Author(s):  
GS Wood ◽  
JS Burke ◽  
S Horning ◽  
RS Doggett ◽  
R Levy ◽  
...  

Abstract Twenty-one cases of non-Hodgkin's lymphoma with cutaneous involvement, other than mycosis fungoides, were evaluated immunologically, histologically, and clinically. Ten patients presented with skin disease alone, seven with concurrent cutaneous and extracutaneous disease, and four with extracutaneous disease only. Twenty of the 21 cases were nonepidermotropic tumors and were equally likely to express B or T phenotypes. None of the cases expressed a true histiocytic phenotype. Almost all cases expressed la and class 1 HLA determinants. Immunophenotypes were stable regardless of time interval, therapy, or body site sampled in seven of eight patients studied serially. In contrast to mycosis fungoides, the T lymphomas exhibited noncerebriform cytology, tumor la expression, lack of mature helper T-cell phenotype, nonepidermotropic histology, a tendency for marrow involvement, and presented as nodules rather than patches or plaques. Since each T lymphoma expressed an abnormal but uniform T-cell phenotype other than mature cytotoxic/suppressor or helper, the neoplastic population could be distinguished from reactive T cells. Reactive elements averaged one- third of the cellular infiltrates and were mainly T cells and macrophages. Langerhans cells were generally normal in number and distribution. Several histopathologic subtypes were identified with diffuse large cell lymphomas, including immunoblastic lymphomas, comprising 71% of cases (15/21). Prediction of the immunophenotype based on cytologic criteria was correct in 67% of cases (14/21). All errors occurred among the 13 high-grade lymphomas. Survival data were consistent with those of prior studies that have indicated that clinical course is dependent on stage and histologic subtype. Non- Hodgkin's cutaneous lymphomas constitute an immunologically, histologically, and clinically heterogeneous group of neoplasms.


2002 ◽  
Vol 76 (2) ◽  
pp. 688-696 ◽  
Author(s):  
Christiane Stahl-Hennig ◽  
Ralph M. Steinman ◽  
Peter Ten Haaft ◽  
Klaus Überla ◽  
Nicole Stolte ◽  
...  

ABSTRACT Deletion of the nef gene from simian immunodeficiency virus (SIV) strain SIVmac239 yields a virus that undergoes attenuated growth in rhesus macaques and offers substantial protection against a subsequent challenge with some SIV wild-type viruses. We used a recently described model to identify sites in which the SIVΔnef vaccine strain replicates and elicits immunity in vivo. A high dose of SIVΔnef was applied to the palatine and lingual tonsils, where it replicated vigorously in this portal of entry at 7 days. Within 2 weeks, the virus had spread and was replicating actively in axillary lymph nodes, primarily in extrafollicular T-cell-rich regions but also in germinal centers. At this time, large numbers of perforin-positive cells, both CD8+ T cells and CD3-negative presumptive natural killer cells, were found in the tonsil and axillary lymph nodes. The number of infected cells and perforin-positive cells then fell. When autopsy studies were carried out at 26 weeks, only 1 to 3 cells hybridized for viral RNA per section of lymphoid tissue. Nevertheless, infected cells were detected chronically in most lymphoid organs, where the titers of infectious virus could exceed by a log or more the titers in blood. Immunocytochemical labeling at the early active stages of infection showed that cells expressing SIVΔnef RNA were CD4+ T lymphocytes. A majority of infected cells were not in the active cell cycle, since 60 to 70% of the RNA-positive cells in tissue sections lacked the Ki-67 cell cycle antigen, and both Ki-67-positive and -negative cells had similar grain counts for viral RNA. Macrophages and dendritic cells, identified with a panel of monoclonal antibodies to these cells, were rarely infected. We conclude that the attenuated growth and protection observed with the SIVΔnef vaccine strain does not require that the virus shift its characteristic site of replication, the CD4+ T lymphocyte. In fact, this immunodeficiency virus can replicate actively in CD4+ T cells prior to being contained by the host, at least in part by a strong killer cell response that is generated acutely in the infected lymph nodes.


Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 464-472 ◽  
Author(s):  
GS Wood ◽  
JS Burke ◽  
S Horning ◽  
RS Doggett ◽  
R Levy ◽  
...  

Twenty-one cases of non-Hodgkin's lymphoma with cutaneous involvement, other than mycosis fungoides, were evaluated immunologically, histologically, and clinically. Ten patients presented with skin disease alone, seven with concurrent cutaneous and extracutaneous disease, and four with extracutaneous disease only. Twenty of the 21 cases were nonepidermotropic tumors and were equally likely to express B or T phenotypes. None of the cases expressed a true histiocytic phenotype. Almost all cases expressed la and class 1 HLA determinants. Immunophenotypes were stable regardless of time interval, therapy, or body site sampled in seven of eight patients studied serially. In contrast to mycosis fungoides, the T lymphomas exhibited noncerebriform cytology, tumor la expression, lack of mature helper T-cell phenotype, nonepidermotropic histology, a tendency for marrow involvement, and presented as nodules rather than patches or plaques. Since each T lymphoma expressed an abnormal but uniform T-cell phenotype other than mature cytotoxic/suppressor or helper, the neoplastic population could be distinguished from reactive T cells. Reactive elements averaged one- third of the cellular infiltrates and were mainly T cells and macrophages. Langerhans cells were generally normal in number and distribution. Several histopathologic subtypes were identified with diffuse large cell lymphomas, including immunoblastic lymphomas, comprising 71% of cases (15/21). Prediction of the immunophenotype based on cytologic criteria was correct in 67% of cases (14/21). All errors occurred among the 13 high-grade lymphomas. Survival data were consistent with those of prior studies that have indicated that clinical course is dependent on stage and histologic subtype. Non- Hodgkin's cutaneous lymphomas constitute an immunologically, histologically, and clinically heterogeneous group of neoplasms.


2019 ◽  
Vol 6 (10) ◽  
pp. A487-495
Author(s):  
Lalit Sharma ◽  
◽  
Kafil Akhtar ◽  
Syed Shamshad Ahmad ◽  
Atia Zakaur Rab ◽  
...  

2018 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Mahdi Asadi ◽  
Aghigh Ziaeemehr ◽  
Soodabeh Shahidsales ◽  
Seyed Amir Aledavood ◽  
Kazem Anvari ◽  
...  

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 623-623
Author(s):  
Giovanna Masci ◽  
Michele Caruso ◽  
Agnese Losurdo ◽  
Piermario Salvini ◽  
Carlo Carnaghi ◽  
...  

623 Background: The overall incidence of male breast cancers (MBC) is around 1% of all breast cancers and is on the rise.Most of our current knowledge regarding its biology and treatment strategies has been extrapolated from its female counterpart. However, from literature data, it is more and more evident that MBC has biological differences compared with female breast cancer (FBC). While hormone receptors are more frequently positive in MBC than in FBC, HER-2 seems to be less expressed in MBC than in FBC, with data ranging from 0 to 18%; no data on Ki-67 have been so far reported. Methods: We retrospectively analyzed the immunohistochemical expression of hormone receptors status, HER-2 protein expression, and Ki-67 in 76 consecutive MBCs, treated within the Humanitas Institutes Network on Cancer Research (INCaRe). HER-2 determinations were carried out according to ASCO/ACP and NEQAS guidelines: cases with score 2+ at IHC were further examined by fluorescent in situ hybridation (FISH). Results: From 2000 to 2011, we treated 76 male breast cases (age 25-87, median 64): 72 patients (94%) had ductal carcinoma and 4 had rare histotypes (2 papillary, 1 mucinous and 1 cribryform). Thirthy-two of 76 patients (42%) had positive axillary lymph-nodes, while 6 (8%) were metastatic at diagnosis. Of these, estrogen receptor and progesterone receptor were positive in 96% and 93% patients respectively; HER-2, evaluable in 67 patients, was positive in 11 (16%). Ki-67 was evaluable in 75 patients and was > 20% in 24 cases (32%), with 20/24 (26%) with Ki-67 > 30%. Grading was evaluable in 65 patients: G1 in 2 (3%), G2 in 41(63%) and G3 in 22 (34%), respectively. Conclusions: In these series, MBC show different patterns from FBC, with some favorable aspects such as higher hormone receptor status and much lower HER-2 expression and some unfavorable features, such as higher Ki-67 values. Although further studies are needed to confirm these data, different treatment strategies would be suggested in MBC than its female counterpart.


Blood ◽  
2011 ◽  
Vol 118 (15) ◽  
pp. 4024-4035 ◽  
Author(s):  
Werner Kempf ◽  
Katrin Pfaltz ◽  
Maarten H. Vermeer ◽  
Antonio Cozzio ◽  
Pablo L. Ortiz-Romero ◽  
...  

AbstractPrimary cutaneous CD30+ lymphoproliferative disorders (CD30+ LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive course, CD30+ LPDs are characterized by an excellent prognosis. Although a broad spectrum of therapeutic strategies has been reported, these have been limited mostly to small retrospective cohort series or case reports, and only very few prospective controlled or multicenter studies have been performed, which results in a low level of evidence for most therapies. The response rates to treatment, recurrence rates, and outcome have not been analyzed in a systematic review. Moreover, international guidelines for staging and treatment of CD30+ LPDs have not yet been presented. Based on a literature analysis and discussions, recommendations were elaborated by a multidisciplinary expert panel of the Cutaneous Lymphoma Task Force of the European Organization for Research and Treatment of Cancer, the International Society for Cutaneous Lymphomas, and the United States Cutaneous Lymphoma Consortium. The recommendations represent the state-of-the-art management of CD30+ LPDs and include definitions for clinical endpoints as well as response criteria for future clinical trials in CD30+ LPDs.


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