Peripheral T-cell lymphomas (PTCL) consist of a heterogeneous group of rare diseases that proliferate from post-thymic clonal lymphocytes. Because they have functional similarities, cell tumors natural killermore commonly called NK cells, were also included in the same group by the World Health Organization in the classification book reviewed in 2016. In the same work, we can highlight a subclassification that includes the types of leukemia (proliferation), streptococcus, Additional and cutaneous streptococcus.
In this range, the journal British Society of Hematology (BSH) published in November 2021 British Journal of Hematology, updated to its recommendations involving the management of these tumors. The evidence classification system used was GRADE SYSTEM. In this article, we will go over the main recommendations. For more details, access the text available in the references.
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General recommendations for LCTPs
Accurate diagnosis of PTCL is very challenging, as high rates of discord can occur between the reported immunophenotypic, cytogenetic, molecular, and morphological markers. It is essential to consult a hematologist experienced in diagnosing these tumors and to review slides whenever possible (GRADE 1B). All PTCLs with nodal presentation show craving in PET/CT and, therefore, should be stratified before and after treatment to assess response and decide on bone marrow transplantation (BMT) signaling in non-leukemic (GRADE 1C) forms. Bone marrow biopsy (BMO) should be offered to all patients because of the low sensitivity of PET/CT in identifying intramedullary disease in these patients, given that 35% of these individuals have bone marrow disease at diagnosis (GRADE 1C).
T-cell leukemia (T-LPL)
LPL-T is a rare disease, accounting for approximately 2% of adult small cell leukemia, with a typical presentation involving peripheral blood lymphocytosis often >100,000 x 109An enlarged spleen and an enlarged gland. The prognosis is cautious. Diagnosis is usually made by analysis of peripheral blood immunophenotype (CD7 positivity and variant positivity for CD4 or CD8) and cytogenetics, with more than half of patients having aberrations on chromosome 8. “Watch and wait” (Watch and Wait) Recommended (Class 1C). First-line treatment with alemtuzumab is recommended in CD52-labeled lymphomas based on a phase II, single-arm study where progression-free survival (PFS), overall survival (OS), overall response rate (GR) and complete response rate (CR) were different. significantly higher than historical controls treated with CHOP-derived regimens (cyclophosphamide, doxorubicin, vincristine, and prednisone) (Grade 1B). If the drug is not available, the latter is the regimen of choice. Consider the use of alemtuzumab in relapsed patients who have not been exposed to the drug or who have an initial response to the regimen with a duration of remission >6 months and where CD52 staining (GRADE 2B) persists. During treatment, provide prophylaxis for pneumonia and herpes zoster, as well as CMV monitoring with quantitative polymerase chain reaction if available (GRADE 1C). The role of BMT is debatable, because data is limited, especially when considering mortality rates associated with the procedure. Therefore, allogeneic bone marrow transplantation should be preferred for young patients and autologous for older patients who are eligible for the procedure (GRADE 2C).
T/NK large granular lymphocytic leukemia (LGL-T / LGL-NK)
LGL-T/NK is diagnosed by peripheral blood/bone marrow immunophenotype and/or BMO immunohistochemistry. It usually appears as clonal lymphocytosis (>six months old) with cytotoxic CD8 and αβ-positive T-cell receptor (TCR) staining. 30% of cases are associated with rheumatic diseases, especially rheumatoid arthritis with Felty’s syndrome. Pancytopenia is often seen in these patients. Asymptomatic patients should be treated “Watch and wait” (Grade 1B). Patients with severe cytopenia, marked neutropenia, and transfusion dependence should be treated with low-dose methotrexate, cyclophosphamide, or oral cyclosporine (Grade 1b). To assess response, treatment should be continued for at least 4 months (Grade 1b). In case of refractory, consider changing the immunosuppressive agent (GRADE 1B).
adult lymphoma/leukemia (ATLL)
This entity is closely related to HTLV-1 infection, and viral or serological DNA research should be performed in patients diagnosed with cancer, first-degree relatives and partners (GRADE 1B). In immunohistochemistry, viral DNA staining is essential to distinguish a virus-carrying patient without disease related to the ATLL infection itself. The prognosis for lymphoma is very reserved, with a median survival of 8 to 10 months. The indolent subspecies has a survival of two to four years. Therefore, allogeneic bone marrow transplantation should be offered as standardization to all patients eligible for the procedure (GRADE 1B). Patients diagnosed with leukemic forms of ATLL should be treated with high doses of zidovudine (AZT) and Interferon-α (IFN-α) as induction, and those who cannot undergo allogeneic BMT as standardization should use maintenance doses. 1 c). Patients with streptococcal forms should receive CHOP + Etoposide (CHOEP) associated with lower doses of AZT + IFN-α (GRADE 1C).
Anaplastic large cell lymphoma (AGCL)
In the latest WHO classification guide published in 2016, four major isoforms are addressed in this group: primary kinase positive (ALK positive), kinase negative (ALK negative), primary cutaneous, and associated with breast implants. Here we will address those that have nothing to do with the skin. In terms of prognosis, ALK-positive LAGC is more favorable, and generally affects younger patients. The International Prognostic Index (IPI) is very useful in stratifying these patients: those with an IPI <2 and/or <40 years are treated as favorable risks. In general, these patients are already advanced stages of diagnosis. The recommendations for early staging are the same as those for advanced staging. The recommended first-line treatment is six cycles of CHP + Brentuximab Vedotin (BV) for ALK-positive and ALK-negative with CD30-positive (GRADE 1A). This data came from the analysis post custom From the ECHELON-2 study due to increased PFS and OS in the study. In the absence of BV, the CHOEP regimen should be preferred. Consider autologous BMT as consolidating into first complete remission in patients with ALK-negative or ALK-positive associated with high risk factors (IPI ≥2 and/or age >40 years) (GRADE 2B). Consider using field radiotherapy + chemotherapy in patients diagnosed with early stage ADCL (GRADE 1B). BV should be attempted for first-line drug-responding patients (CD 30+) with high response rates and for relapsed/resistant patients not previously exposed to the drug (GRADE 1B). Anaplastic lymphomas related to breast implants are generally treated with a surgical approach and removal of the implant when it is localized.
Unspecified PTCL and Immunovascular Lymphoma
Unfortunately, again, we are faced with a subset of diseases with poor prognosis, with a 5-year treatment-failure survival approaching 20% and a 5-year OS of 30%. IPI can be used as an outcome indicator, but little progress has been observed in treatments in recent years. The first line is still a CHOP-based system (GRADE 1B) and the data is inconclusive to indicate CHOEP. In the ECHELON-2 study, the fraction of patients with these specific diagnoses of CD30 was small and it was not possible to indicate the use of BV, which has not yet been approved for these purposes in Europe. Self-consolidating BMT after first-line response (GRADE 2B) should be considered. Rescue should be attempted with high doses of chemotherapy in relapsed/refractory patients (GRADE 1C). Prevention of central nervous system (CNS) infiltration should be based on the CNS-IPI grade of diffuse large B-cell lymphoma (GRADE 2C). Allogeneic bone marrow transplantation should be offered for relapses/refractory after a second remission (grade 2c).
Additional T/NK . Nodular Lymphoma
Aggressive extranodal lymphoma, usually NK type, with CD2+, CD56+, CD3+, CD8 cytotoxic markers). Epstein-Barr virus (EBV) is involved in all cases. Classically it appears in the nasal cavity local or systemic. The presence of disease outside the nose It is the main factor for poor prognosis. The index used for diagnosis is the “PINK” index. Since the treatment of localized and systemic forms is different, correct diagnosis and staging of treatment is necessary. PET/CT should be offered to evaluate for extranasal disease (GRADE 1B). Magnetic resonance imaging (MRI) should be performed to assess the extent of local disease (GRADE 2C). The presence of EBV in cancer cells must be confirmed using the EBER-ISH (small in situ hybridization) technique. The RNAs encoded were mapped to EBV (GRADE 1A). First-line treatment includes chemotherapy with anthracycline, platinum, and/or L-asparaginase-free regimens such as SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide), DDGP, or AspMetDex with 6-7 treatment-related deaths %, 5 years SG 50% and RG rate 80% (GRADE 1B). For patients with localized disease (stage I and II), perform radiotherapy with respective areas associated with chemotherapy (GRADE 1B). The role of BMT has been poorly defined, however, and autologous bone marrow transplantation in the first fusion is suggested.
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Hepato-splenic T-cell lymphoma
Rare, aggressive lymphoma that mostly affects young adults and may be secondary to solid organ transplantation or the use of immunosuppressants in other clinical settings. It is a systemic extraauricular disease that affects the liver, spleen, and bone marrow. Typical histology is characterized by tumor cells infiltrating the splenic sinusoidal capillaries. The classic phenotype expresses a gamma delta TCR with isochromosome 7q abnormalities. An alpha-beta variant can be found. PET/CT confirms the absence of lymphadenopathy. Trisomy 8 is seen classically. Data to evaluate the best treatment is scarce, but promising results are reported in case series using high-dose chemotherapy such as IVAC/ICE as induction (GRADE 2C) and allogeneic BMT as standardization (GRADE 1C).
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