Cutaneous T-cell lymphomas, which include mycosis fungoides and Sézary syndrome, are neoplasias of malignant T lymphocytes that usually possess the helper/inducer cell surface phenotype and initially present as skin involvement.[1] Cutaneous T-cell lymphomas should be distinguished from other T-cell lymphomas that involve the skin, such as anaplastic large cell lymphoma (CD30 positive), peripheral T-cell lymphoma (CD30 negative, with no epidermal involvement), or adult T-cell leukemia/lymphoma (usually with systemic involvement).[2,3] For more information about these types of T-cell lymphomas, see Peripheral T-Cell Non-Hodgkin Lymphoma Treatment.
Typically, the natural history of cutaneous T-cell lymphoma is indolent.[4] Symptoms of the disease may be present for long periods, in a range of 2 to 10 years, because cutaneous eruptions wax and wane before being confirmed by biopsy. Cutaneous T-cell lymphomas are treatable with available topical therapy, systemic therapy, or both. Curative modalities have proven elusive, with the possible exception of patients with minimal disease confined to the skin.
In addition, several benign or indolent conditions can be confused with mycosis fungoides. It is important to consult with a pathologist who has expertise in distinguishing these conditions.[1]
The prognosis of patients with cutaneous T-cell lymphomas is based on the extent of disease (stage) at presentation.[5] The presence of lymphadenopathy and involvement of peripheral blood and viscera increase in likelihood with worsening cutaneous involvement and define poor prognostic groups.[5-8] The Cutaneous Lymphoma International Consortium retrospectively reviewed 1,275 patients and found that the following four independent prognostic markers indicate a worse survival:[9]
The median survival following diagnosis varies according to stage. Patients with stage IA disease have a median survival of 20 years or more. Most deaths for this group are not caused by, nor are they related to, mycosis fungoides.[10,11] In contrast, more than 50% of patients with stage III through stage IV disease die of mycosis fungoides, with a median survival of approximately 5 years.[7,9,12,13] The Cutaneous Lymphoma International Prognostic index used male sex, age older than 60 years, plaques, lymph nodes, blood involvement, and visceral involvement as poor prognostic factors to define predicted overall survival (OS) and progression-free survival in both early-stage and advanced-stage groups.[14]
A report on 1,798 patients from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program database found an increase in second malignancies in patients with mycosis fungoides (standardized incidence ratio, 1.32; 95% confidence interval [CI], 1.15–1.52), especially for Hodgkin lymphoma, non-Hodgkin lymphoma, and myeloma.[15] Another report on 4,459 patients from the SEER database found that the 19.2% of African American patients with mycosis fungoides had shorter OS, potentially attributable to disease characteristics, socioeconomic status, and type of therapy (hazard ratio, 1.47; 95% CI, 1.25–1.74; P < .001).[16]
Cutaneous disease can manifest as an eczematous patch or plaque stage covering less than 10% of the body surface (T1), a plaque stage covering 10% or more of the body surface (T2), or as tumors (T3) that frequently undergo necrotic ulceration.[17,18] Several retrospective studies showed that 20% of patients have disease that progresses from stage I or II to stage III or IV.[19-21] Sézary syndrome presents with generalized erythroderma (T4) and peripheral blood involvement. However, there is some disagreement about whether mycosis fungoides and Sézary syndrome are actually variants of the same disease.[22] The same retrospective study with a median follow-up of 14.5 years found that only 3% of 1,422 patients progressed from mycosis fungoides to Sézary syndrome.[19]
There is consensus that patients with Sézary syndrome (leukemic involvement) have a poor prognosis (median survival, 4 years), with or without the typical generalized erythroderma.[23,24] Cytologic transformation from a low-grade lymphoma to a high-grade lymphoma (large cell transformation) occurs rarely (<5%) during the course of these diseases and is associated with a poor prognosis.[25-27] A retrospective analysis of 100 cases with large cell transformation found reduced disease-specific survival with extracutaneous transformation, increased extent of skin lesions, and CD30 negativity.[28] A common cause of death during the tumor phase is septicemia caused by chronic skin infection with staph species, herpes simplex, herpes zoster, and fungal skin infections.[29,30]
Folliculotropic mycosis fungoides is a variant of mycosis fungoides marked by folliculotropic, rather than epidermotropic, neoplastic infiltrates, with preferential location in the head and neck area.[31] Early plaque-stage folliculotropic mycosis fungoides have a very indolent prognosis, while extracutaneous disease portends a very poor prognosis.[31]
The histological diagnosis of mycosis fungoides and other cutaneous T-cell lymphomas is usually difficult to determine in the initial stages of the disease and may require the review of multiple biopsies by an experienced pathologist.
A definitive diagnosis from a skin biopsy requires the presence of cutaneous T-cell lymphoma cells (convoluted lymphocytes), a band-like upper dermal infiltrate, and epidermal infiltrations with Pautrier abscesses (collections of neoplastic lymphocytes). A definitive diagnosis of Sézary syndrome may be made from a peripheral blood evaluation when skin biopsies are consistent with the diagnosis. Supportive evidence for circulating Sézary cells is provided by T-cell receptor gene analysis, identification of the atypical lymphocytes with hyperconvoluted or cerebriform nuclei, and flow cytometry with the characteristic deletion of cell surface markers such as CD7 and CD26. However, none of these is individually pathognomonic for lymphoma.[1,2]
The American Joint Committee on Cancer (AJCC) has designated staging by TNM (tumor, node, metastasis) classification to define cutaneous T-cell lymphomas.[1] Peripheral blood involvement with cutaneous T-cell lymphoma cells is correlated with more advanced skin stage, lymph node and visceral involvement, and shortened survival.
Cutaneous T-cell lymphomas also have a formal staging system proposed by the International Society for Cutaneous Lymphomas and the European Organisation for Research and Treatment of Cancer.[2,3]
| EORTC Classification | Dutch System | NCI-VA Classification |
|---|---|---|
| DL = dermatopathic lymphadenopathy; EORTC = European Organisation for Research and Treatment of Cancer; LN = lymph nodes; N = regional lymph node; NCI = National Cancer Institute; VA = U.S. Department of Veterans Affairs. | ||
| aReprinted with permission from AJCC: Primary Cutaneous Lymphomas. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 967–72. | ||
| N1 | Grade 1: DL | LN0: No atypical lymphocytes. |
| LN1: Occasional and isolated atypical lymphocytes (not arranged in clusters). | ||
| LN2: Many atypical lymphocytes or lymphocytes in 3-6‒cell clusters. | ||
| N2 | Grade 2: DL; early involvement by mycosis fungoides (presence of cerebriform nuclei <7.5 µm [micrometer]). | LN3: Aggregates of atypical lymphocytes; nodal architecture preserved. |
| N3 | Grade 3: Partial effacement of lymph node architecture; many atypical cerebriform mononuclear cells. | LN4: Partial/complete effacement of nodal architecture by atypical lymphocytes or frankly neoplastic cells. |
| Grade 4: Complete effacement. | ||
| Stage | TNM | Description | B | Peripheral Blood Involvement Criteria |
|---|---|---|---|---|
| T = primary tumor; N = regional lymph node; M = distant metastasis; B = peripheral blood involvement. | ||||
| aReprinted with permission from AJCC: Primary Cutaneous Lymphomas. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 967–72. | ||||
| The explanations for superscripts b through f are at the end of Table 5. | ||||
| IA | T1, N0, M0 | T1 = Limited patches,b papules, and/or plaquesc covering <10% of the skin surface. | B0,1 | B0 = Absence of significant blood involvement: ≤5% of peripheral blood lymphocytes are atypical (Sézary) cells.d |
| –T1a = T1a (patch only). | ||||
| –T1b = T1b (plaque ± patch). | –B0a = Clone negativee | |||
| –B0b = Clone positivee | ||||
| N0 = No clinically abnormal peripheral lymph nodes;f biopsy not required. | B1 = Low blood tumor burden: >5% of peripheral blood lymphocytes are atypical (Sézary) cells, but does not meet the criteria of B2. | |||
| M0 = No visceral organ involvement. | –B1a = Clone negativee | |||
| –B1b = Clone positivee | ||||
| IB | T2, N0, M0 | T2 = Patches, papules, or plaques covering ≥10% of the skin surface. | B0,1 | See B0, B1 descriptions above in this table, Stage IA. |
| –T2a = T2a (patch only). | ||||
| –T2b = T2b (plaque ± patch). | ||||
| N0 = No clinically abnormal peripheral lymph nodes;f biopsy not required. | ||||
| M0 = No visceral organ involvement. | ||||
| Stage | TNM | Description | B | Peripheral Blood Involvement Criteria |
|---|---|---|---|---|
| T = primary tumor; N = regional lymph node; M = distant metastasis; B = peripheral blood involvement; LN = lymph nodes; NCI = National Cancer Institute. | ||||
| aReprinted with permission from AJCC: Primary Cutaneous Lymphomas. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 967–72. | ||||
| The explanations for superscripts e through g are at the end of Table 5. | ||||
| IIA | T1,2; N1,2; M0 | See T1–2 descriptions above in Table 2, Stages IA, IB. | B0,1 | See B0, B1 descriptions above in Table 2, Stage IA. |
| N1 = Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0–2. | ||||
| –N1a = Clone negative.e | ||||
| –N1b = Clone positive.e | ||||
| N2 = Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. | ||||
| –N2a = Clone negative.e | ||||
| –N2b = Clone positive.e | ||||
| M0 = No visceral organ involvement. | ||||
| IIB | T3, N0–2, M0 | T3 = One or more tumorsg (≥1 cm in diameter). | B0,1 | See B0, B1 descriptions above in Table 2, Stage IA. |
| –T3a = Multiple lesions involving 2 noncontiguous body regions. | ||||
| –T3b = Multiple lesions involving ≥3 body regions. | ||||
| N0 = No clinically abnormal peripheral lymph nodes;f biopsy not required. | ||||
| See N1–2 descriptions above in this table, Stage IIA | ||||
| M0 = No visceral organ involvement. | ||||
| Stage | TNM | Description | B | Peripheral Blood Involvement Criteria |
|---|---|---|---|---|
| T = primary tumor; N = regional lymph node; M = distant metastasis; B = peripheral blood involvement. | ||||
| aReprinted with permission from AJCC: Primary Cutaneous Lymphomas. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 967–72. | ||||
| III | T4, N0–2, M0 | T4 = Confluence of erythema covering ≥80% of body surface area. | B0,1 | See B0, B1 descriptions above in Table 2, Stage IA. |
| See N0–2 descriptions above in Table 3, Stages IIA, IIB. | ||||
| M0 = No visceral organ involvement. | ||||
| IIIA | T4, N0–2, M0 | T4 = Confluence of erythema covering ≥80% of body surface area. | B0 | See B0 description above in Table 2, Stage IA. |
| See N0–2 descriptions above in Table 3, Stages IIA, IIB. | ||||
| M0 = No visceral organ involvement. | ||||
| IIIB | T4, N0–2, M0 | T4 = Confluence of erythema covering ≥80% of body surface area. | B1 | See B1 description above in Table 2, Stage IA. |
| See N0–2 descriptions above in Table 3, Stages IIA, IIB. | ||||
| M0 = No visceral organ involvement. | ||||
| Stage | TNM | Description | B | Peripheral Blood Involvement Criteria |
|---|---|---|---|---|
| T = primary tumor; N = regional lymph node; M = distant metastasis; B = peripheral blood involvement; LN = lymph nodes; NCI = National Cancer Institute. | ||||
| aReprinted with permission from AJCC: Primary Cutaneous Lymphomas. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 967–72. | ||||
| bFor skin, patch indicates any size skin lesion without significant elevation or induration. Presence/absence of hypo- or hyperpigmentation, scale, crusting, and/or poikiloderma should be noted. | ||||
| cFor skin, plaque indicates any size skin lesion that is elevated or indurated. Presence/absence of scale, crusting, and/or poikiloderma should be noted. Histological features such as folliculotropism, large cell transformation (>25% large cells) and CD30 positivity or negativity, as well as clinical features such as ulceration, are important to document. | ||||
| dFor blood, Sézary cells are defined as lymphocytes with hyperconvoluted cerebriform nuclei. If Sézary cells cannot be used to determine tumor burden for B2, then one of the following modified ISCL criteria, along with a positive clonal rearrangement of the T-cell receptor (TCR), may be used instead: (1) expanded CD4+ or CD3+ cells with a CD4/CD8 ratio of >10, or (2) expanded CD4+ cells with abnormal immunophenotype, including loss of CD7 (>40%) or CD26 (>30%). | ||||
| eA T-cell clone is defined by polymerase chain reaction or Southern blot analysis of the TCR gene. | ||||
| fFor node, abnormal peripheral lymph node(s) indicates any palpable peripheral node that on physical examination is firm, irregular, clustered, fixed or ≥1.5 cm in diameter. Node groups examined on physical examination include cervical, supraclavicular, epitrochlear, axillary, and inguinal. Central nodes, which generally are not amenable to pathological assessment, currently are not considered in the nodal classification unless used to establish N3 histopathologically. | ||||
| gFor skin, tumor indicates at least one 1-cm diameter solid or nodular lesion with evidence of depth and/or vertical growth. Note the total number of lesions, total volume of lesions, largest size lesion, and region of body involved. Also note whether there is histological evidence of large cell transformation. Phenotyping for CD30 is encouraged. | ||||
| hFor viscera, spleen and liver may be diagnosed by imaging criteria. | ||||
| IVA1 | T1–4, N0–2, M0 | See T1‒2 descriptions above in Table 2, Stages IA, IB. | B2 | B2 = High blood tumor burden: ≥1,000 mcg/L Sézary cellsd or >40% CD4+/CD7- or increased >30% CD4+/CD26- cells with positive clone.e |
| T3 = One or more tumorsg (≥1 cm in diameter). | ||||
| –T3a = Multiple lesions involving 2 noncontiguous body regions. | ||||
| –T3b = Multiple lesions involving ≥3 body regions. | ||||
| T4 = Confluence of erythema covering ≥80% of body surface area. | ||||
| See N0–2 descriptions above in Table 3, Stages IIA, IIB. | ||||
| M0 = No visceral organ involvement. | ||||
| IVA2 | T1–4, N3, M0 | See T1‒2 descriptions above in Table 2, Stages IA, IB and see T3–4 descriptions above in this table, Stage IVA1. | B0–2 | See B0, B1 descriptions above in Table 2, Stage IA and see B2 description above in this table, Stage IVA1. |
| N3 = Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3–4 or NCI LN4; clone positive or negative. | ||||
| M0 = No visceral organ involvement. | ||||
| IVB | T1–4, N0–3, M1 | See T1‒2 descriptions above in Table 2, Stages IA, IB and see T3–4 descriptions above in this table, Stage IVA1. | B0–2 | See B0, B1 descriptions above in Table 2, Stage IA and see B2 description above in this table, Stage IVA1. |
| See N0–2 descriptions above in Table 3, Stages IIA, IIB. | ||||
| N3 = Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3–4 or NCI LN4; clone positive or negative. | ||||
| M1 = Visceral involvement (must have pathology confirmation,h and organ involved should be specified). | ||||
Anecdotal responses, some lasting for months, can be seen with aggressive antibiotic treatment of Staphylococcus aureus, with corresponding decreased expression of interleukin-2 receptors, STAT signaling, and T-cell proliferation.[1][Level of evidence C3]
These types of treatments produce remissions, but long-term remissions are uncommon. Therefore, treatment is considered palliative for most patients, although major symptomatic improvement is regularly achieved. Survival in excess of 8 years is common for patients with early stages of disease. All patients with cutaneous T-cell lymphomas are candidates for clinical trials evaluating new approaches to treatment.
| Stage (TNM Definitions) | Treatment Options |
|---|---|
| Stage I and Stage II Mycosis Fungoides | Photodynamic therapy |
| Radiation therapy | |
| Biological therapy | |
| Chemotherapy | |
| Other drug therapy | |
| Targeted therapy | |
| Stage III and Stage IV Mycosis Fungoides and Sézary Syndrome | Photodynamic therapy |
| Radiation therapy | |
| Biological therapy | |
| Chemotherapy | |
| Other drug therapy | |
| Targeted therapy | |
| Checkpoint inhibitors | |
| Recurrent Mycosis Fungoides and Sézary Syndrome | Radiation therapy |
| Photodynamic therapy | |
| Chemotherapy | |
| Other drug therapy | |
| Biological therapy | |
| Allogeneic stem cell transplant | |
| Targeted therapy | |
| Primary Cutaneous Anaplastic Large Cell Lymphoma | Radiation therapy |
| Targeted therapy | |
| Chemotherapy | |
| Subcutaneous Panniculitis-Like T-Cell Lymphoma | Immunosuppression |
| Chemotherapy | |
| Targeted therapy | |
| Allogeneic stem cell transplant | |
| Primary Cutaneous Gamma Delta T-Cell Lymphoma | Chemotherapy |
| Allogeneic stem cell transplant | |
| Primary Cutaneous Aggressive Epidermotropic CD8-Positive T-Cell Lymphoma | Chemotherapy |
| Allogeneic stem cell transplant |
Several forms of treatment can produce complete resolution of skin lesions in this stage, so the choice of therapy is dependent on local expertise and the facilities available. With therapy, the survival of patients with stage IA disease can be expected to be the same as for age- and sex-matched controls.[1-3]
There is no curative therapy and no clear difference in overall survival (OS) among the treatment options for patients with stage I and stage II mycosis fungoides.
A randomized study of 103 patients compared combined total-skin electron-beam radiation (TSEB) plus combination chemotherapy with sequential topical therapies.[4] In the latter group, combination chemotherapy was reserved for patients with symptomatic extracutaneous disease or disease that was refractory to topical therapies. Patients with any disease stage were eligible. Although the complete response rate was higher with combined therapy, toxic effects were considerably greater, and no difference was seen in disease-free survival (DFS) or OS between the two groups.[4][Level of evidence A1]
Treatment options for stages I and II mycosis fungoides include the following:[5]
Chemotherapeutic agents generally demonstrate short durations of response. In a retrospective review of 198 patients with advanced-stage disease, the median TTNT was 4 months.[23] However, these comparisons may be confounded by the order in which the agents were introduced.
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Mycosis Fungoides
There is no curative therapy and no clear difference in overall survival (OS) among the treatment options for patients with stage III and stage IV disease.
The use of single alkylating agents has produced objective responses in 60% of patients, with a duration of less than 6 months. One of the alkylating agents (e.g., mechlorethamine, cyclophosphamide, or chlorambucil), or the antimetabolite methotrexate is the most frequently used. Single agents have not cured any patients, and insufficient data exist to determine whether these agents prolong survival. Combination chemotherapy is not definitely superior to single agents. Even in patients with stage IV disease, treatments directed at the skin may provide significant palliation.
A randomized study of 103 patients compared combined total-skin electron-beam radiation (TSEB) plus combination chemotherapy with conservation therapy consisting of sequential topical therapies.[1] In the latter group, combination chemotherapy was reserved for patients with symptomatic extracutaneous disease or for disease that was refractory to topical therapies. Patients with any disease stage were eligible. Although the complete response rate was higher with combined therapy, toxic effects were considerably greater, and no difference was seen in disease-free survival (DFS) or OS between the two groups.[1][Level of evidence A1]
Sézary Syndrome
Sézary syndrome is a rare leukemic variant of cutaneous T-cell lymphoma characterized by erythroderma, circulating Sézary cells with cerebriform nuclei, lymphadenopathy, and pruritus.[2] This condition typically progresses rapidly with only short duration of response to most therapies. A retrospective review of 176 patients with Sézary syndrome identified the following poor prognostic factors:[3]
Remissions attained by using extracorporeal photophoresis, interferon alfa, or retinoids may be followed by allogeneic stem cell transplant. In an anecdotal series of 16 patients with Sézary syndrome after allogeneic transplant, 9 were in complete remission after 4 years.[4]
Treatment options for stages III and IV mycosis fungoides and Sézary syndrome include the following (note that in this clinical setting, the skin is easily injured; any of the topical therapies must be administered with extreme caution):[2,5]
Chemotherapeutic agents generally demonstrate short durations of response. In a retrospective review of 198 patients with advanced-stage disease, the median TTNT was 4 months.[29] However, these comparisons may be confounded by the order in which the agents were introduced.
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
The treatment of patients with relapsed mycosis fungoides and Sézary syndrome involves the joint decisions of the dermatologist, medical oncologist, and radiation oncologist. It may be possible to re-treat localized areas of relapse in the skin with additional electron-beam radiation therapy or to repeat total-skin electron-beam radiation therapy (TSEB).[1] Photon radiation to bulky skin or nodal masses may prove beneficial. If these options are not possible, then continued topical treatment with other modalities such as mechlorethamine or psoralen and ultraviolet A radiation (PUVA) may be warranted to relieve cutaneous symptoms.
Patients should consider clinical trials as a therapeutic option.
Treatment options under clinical evaluation for recurrent mycosis fungoides and Sézary syndrome include the following:[2,3]
Mogamulizumab is often avoided in patients scheduled to undergo allogeneic SCT, based on data from a Japanese study that showed an increased risk of severe graft-versus-host disease (GVHD) in patients treated with mogamulizumab beforehand.[49] The relevance of these findings in other countries and the impact of different GVHD prophylaxis regimens in these patients remains to be determined.
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Primary cutaneous anaplastic large cell lymphoma presents in the skin only with no preexisting lymphoproliferative disease and no extracutaneous sites of involvement.[1-3] Patients with this type of lymphoma have disease ranging from clinically benign lymphomatoid papulosis, marked by localized nodules that may regress spontaneously, to progressive and systemic illness requiring aggressive doxorubicin-based combination chemotherapy. This spectrum has been called the primary cutaneous CD30-positive T-cell lymphoproliferative disorder.
Patients with localized disease usually undergo radiation therapy. With more disseminated involvement, watchful waiting or doxorubicin-based combination chemotherapy is used.[1-3]
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is localized to subcutaneous tissue and can be associated with hemophagocytic lymphohistiocytosis (HLH).[1-4] Anecdotal reports suggest that the presence or absence of HLH is an important prognostic indicator.[5] Patients with SPTCL have cells that express alpha-beta phenotype.
Patients with gamma-delta phenotype have a more aggressive clinical course that is instead classified as primary cutaneous gamma-delta T-cell lymphoma.[6-8] For more information, see Peripheral T-Cell Non-Hodgkin Lymphoma Treatment.
The management of SPTCL depends on the clinical presentation—including the severity of symptoms, cytopenias, and the presence or absence of HLH—and the apparent disease trajectory and aggressiveness. Indolent or smoldering forms of SPTCL are often treated with immunosuppression, including oral methotrexate [9] or cyclosporine.[10] In contrast, aggressive forms are frequently treated with combination chemotherapy such as CHO(E)P (cyclophosphamide, doxorubicin, vincristine, and prednisone with or without etoposide) with variable responses per anecdotal reports.[11] The JAK2 inhibitor ruxolitinib has also been used in SPTCL with associated HLH, including anecdotal reports of responses in patients with disease that did not respond to CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) therapy.[12] In cases of particularly aggressive or relapsed disease, consolidation with allogeneic stem cell transplant has also been used.[13,14]
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Primary cutaneous gamma-delta T-cell lymphoma (PCGDTCL) is a rare and extremely aggressive form of cutaneous T-cell lymphoma associated with a poor prognosis. These patients may manifest involvement of the epidermis, dermis, subcutaneous region, or mucosa with or without ulceration. PCGDTCL is treated similarly to the most aggressive peripheral T-cell lymphomas, with CHO(E)P (cyclophosphamide doxorubicin, vincristine, and prednisone with or without etoposide).[1-5] For patients achieving remission, there are reports of prolonged survival following consolidation with allogeneic stem cell transplant.[6]
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Primary cutaneous aggressive epidermotropic CD8-positive T-cell lymphoma is another rare and especially aggressive form of cutaneous T-cell lymphoma. Patients typically present with ulcerative plaques or tumors, and mucosal involvement is common. Neoplastic cells are characterized by expression of CD8 and a cytotoxic phenotype.Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation. Multimodal chemotherapy, such as CHO(E)P (cyclophosphamide, doxorubicin, vincristine, and prednisone with or without etoposide), is often used. Outcomes are generally poor, and prolonged remissions are extremely uncommon in the absence of allogeneic stem cell transplant.[1]
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
These references have been identified by members of the PDQ Adult Treatment Editorial Board as significant in the field of mycosis fungoides and other cutaneous T-cell lymphoma treatment. This list is provided to inform users of important studies that have helped shape the current understanding of and treatment options for MF/SS. Listed after each reference are the sections within this summary where the reference is cited.
Cited in:
Cited in:
Cited in:
Cited in:
Cited in:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Stage Information for Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas
Revised Table 5, Definitions of TNM Stages IVA1, IVA2, and IVB to include peripheral blood involvement criteria for B2.
Treatment Option Overview for Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas
Revised Table 6, Treatment Options for Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas.
Treatment of Stage III and Stage IV Mycosis Fungoides and Sézary Syndrome
Added text to state that there are anecdotal reports of hyperprogression of T-cell malignancies following treatment with immune checkpoint inhibitors (cited Ratner et al. and Bennani et al. as references 53 and 54, respectively).
Treatment of Recurrent Mycosis Fungoides and Sézary Syndrome
Added text to state that mogamulizumab appeared to be especially effective in patients with blood involvement, such as those with Sézary syndrome.
Added text to state that mogamulizumab is often avoided in patients scheduled to undergo allogeneic stem cell transplant, based on data from a Japanese study (cited Sugio et al. as reference 49).
Treatment of Subcutaneous Panniculitis-Like T-Cell Lymphoma
This section was extensively revised.
Treatment of Primary Cutaneous Gamma-Delta T-Cell Lymphoma
Added this new section.
Treatment of Primary Cutaneous Aggressive Epidermotropic CD8-Positive T-Cell Lymphoma
Added this new section.
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of mycosis fungoides and other cutaneous T-cell lymphomas. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
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The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389288]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.
Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
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