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T-Cell Lymphoblastic Lymphoma

Precursor T Lymphoblastic Lymphoma

T-cell lymphoblastic lymphoma is a rare lymphoproliferative disorder and a subtype of adult non-Hodgkin lymphoma. Both solid tumors in lymphatic tissues as well as impairment of bone marrow function may be presented by affected individuals, and, in recent years, survival rates have increased significantly due to improved therapeutic regimens.

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Presentation

The majority of T-LBL patients presents with complaints resulting from a space-occupying mediastinal mass that may assume considerable dimensions upon diagnosis. In fact, this mediastinal mass often provokes bilateral pleural effusion and pericardial effusion, which, in turn, may cause dyspnea, cough, chest pain, attenuation of breath sounds and impaired resonance as assessed by means of percussion. Eventually, affected individuals may develop superior vena cava syndrome. The latter comprises distension of neck veins and raised jugular venous pressure as well as facial edema. The second most common site of solid tumor development in T-LBL patients are cervical, supraclavicular, and axillary lymph nodes. Tumor growth in non-lymphatic tissues is not characteristic of early T-LBL. If observed, liver and spleen are primarily affected. Neoplasms of the skin, tumors affecting the head and neck region, the bones, kidneys or gonads have only occasionally been noted in this context [10]. However, disease progression increases the risk of central nervous system compromise, and this organ system is usually the first site of relapse. Furthermore, the degree of bone marrow involvement seems to correlate with the incidence of lymphoma-associated brain and spinal cord tumors. Bone marrow involvement is detected upon diagnosis in about two thirds of T-LBL patients, and bone marrow blast counts are below 25%. Of note, T-LBL patients may present with multiple tumors, e.g., a mediastinal mass may be accompanied by neoplastic lymphadenopathy. Patients may additionally present constitutional symptoms like fatigue, fever, night sweats and weight loss.

Easy Bruising
  • Thrombocytopenia is a condition when there is a low number of platelets which can cause bleeding and easy bruising with no apparent cause. Low numbers of all three blood cell counts is called pancytopenia.[lls.org]
Cervical Lymphadenopathy
  • Herein, we describe such a case in a 26-year-old woman who presented with progressive cervical lymphadenopathy.[ncbi.nlm.nih.gov]
Abdominal Lymphadenopathy
  • Staging investigations revealed mediastinal and abdominal lymphadenopathy, bilateral pleural effusions and bone marrow infiltration, in keeping with stage IVB disease.[ncbi.nlm.nih.gov]
Lymphadenopathy
  • This was followed by the second episode of spontaneous tumor lysis syndrome and spontaneous regression of lymphadenopathy again.[ncbi.nlm.nih.gov]
  • This case suggests that in the immunocompromised patients who had history of lymphoma, infectious etiology should be ruled out for PET scan-positive lymphadenopathy.[ncbi.nlm.nih.gov]
  • We have described a 20-year-old man with a non-T-cell lymphoblastic lymphoma manifested by extensive osteolytic lesions and hypercalcemia (serum calcium value of 13.5 mg/dL), without lymphadenopathy.[ncbi.nlm.nih.gov]
  • T-cell lymphoblastic lymphoma (T-LBL) is a highly aggressive lymphoma characterized by precursor T-cell malignancy and lymphadenopathy or mediastinal involvement.[ncbi.nlm.nih.gov]
  • Two months after treatment, the patient showed near complete remission of the lymphadenopathy. These data suggest a possible synergistic effect with bortezomib in combination with doxorubincin and dexamethasone.[ncbi.nlm.nih.gov]
Fever
  • We report the simultaneous occurrence of T-cell lymphoblastic lymphoma (T-LBL) and WT in an 81-year-old patient, who presented with fever, night sweats and enlargement of the right parotid gland.[ncbi.nlm.nih.gov]
  • Keywords: Lymphoma, Bone marrow, Lymph node, Immunohistochemistry Abstract A 16-year-old girl presented to the out patient department in Grande International Hospital with complaints of fever since 3 weeks, tonsillitis and multiple lymphadenopathy.[nepjol.info]
  • Patients may additionally present constitutional symptoms like fatigue, fever, night sweats and weight loss. Diagnostic imaging is of major importance to evaluate which organs are compromised by the disease.[symptoma.com]
  • Other symptoms include fever, weight loss, fatigue, and/or night sweats. Non-Hodgkin lymphoma ( NHL ) is a large group of cancers derived from lymphocytes (white blood cells).[cibmtr.org]
  • […] platelets means a compromised ability to form clots, so patients may develop bruises or bloody noses; and (3) impaired production of certain white blood cells, especially neutrophils, can be associated with increased infections that manifest as high fevers[cumc.columbia.edu]
Weight Loss
  • Advances in Hematologic Malignancies Issue 1, Spring 2013 Figure 1 A 41-year-old man presented with 4 weeks of progressive dysphagia, reflux, involuntary weight loss, night sweats, dyspnea on exertion, and an enlarging left neck mass.[dana-farber.org]
  • Patients may additionally present constitutional symptoms like fatigue, fever, night sweats and weight loss. Diagnostic imaging is of major importance to evaluate which organs are compromised by the disease.[symptoma.com]
  • Other symptoms include fever, weight loss, fatigue, and/or night sweats. Non-Hodgkin lymphoma ( NHL ) is a large group of cancers derived from lymphocytes (white blood cells).[cibmtr.org]
  • Symptoms include swollen lymph nodes, fatigue, weight loss, and night sweats . Diagnosis and Staging of Lymphoblastic Lymphoma To accurately diagnose and stage this disease, doctors may use blood work and bone marrow or lymph node biopsies.[lymphomainfo.net]
Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019[patientslikeme.com]
  • Patients may additionally present constitutional symptoms like fatigue, fever, night sweats and weight loss. Diagnostic imaging is of major importance to evaluate which organs are compromised by the disease.[symptoma.com]
  • They include: Fatigue Fever Loss of appetite or weight Night sweats Many symptoms of acute lymphoblastic leukemia are the result of a shortage of normal blood cells. That's because leukemia cells crowd out these normal cells in the bone marrow.[webmd.com]
  • Other symptoms include fever, weight loss, fatigue, and/or night sweats. Non-Hodgkin lymphoma ( NHL ) is a large group of cancers derived from lymphocytes (white blood cells).[cibmtr.org]
  • Symptoms include swollen lymph nodes, fatigue, weight loss, and night sweats . Diagnosis and Staging of Lymphoblastic Lymphoma To accurately diagnose and stage this disease, doctors may use blood work and bone marrow or lymph node biopsies.[lymphomainfo.net]
Anemia
  • For patients accompanying with anemia, bulky mass and superior vena cava syndrome, their prognosis is worse.[ncbi.nlm.nih.gov]
  • Emperipolesis by megakaryocytes in patients with non-Hodgkin's lymphoma and megaloblastic anemia. J Submicrosc Cytol1982;14(2):407-413. 3. Takeya M, Takahashi K.[cogprints.org]
  • Anemia is a condition when there is a low number of red cells in the blood which can cause fatigue and shortness of breath.[lls.org]
  • A shortage of red blood cells may cause symptoms of anemia, including: Fatigue or weakness Dizziness Feeling cold Light-headedness Shortness of breath A shortage of normal white blood cells may result in: Fevers Recurring infections A shortage of blood[webmd.com]
Pleural Effusion
  • RESULTS: G-banding and spectral karyotyping of pleural effusion cells showed 47,XY,dup(1)(q21q32),t(7;9)(q34;q34), 20.[ncbi.nlm.nih.gov]
  • A 3-year-old girl presenting with a huge mediastinal tumor and massive pleural effusion, was diagnosed with stage III γδ T-cell lymphoblastic lymphoma (γδ T-LBL) by flow cytometry of effusion cells.[ncbi.nlm.nih.gov]
  • Immunologic studies of the surface phenotype of the malignant cells in a pleural effusion with monoclonal antibodies revealed the cells to be of thymic origin and distinguished them from the surface phenotypes seen in T-cell acute lymphoblastic leukemia[ncbi.nlm.nih.gov]
  • This report describes a case of post-transplantation lymphoma presenting with a mediastinal mass causing superior vena cava syndrome, tracheal obstruction, and pleural effusion.[ncbi.nlm.nih.gov]
  • Staging investigations revealed mediastinal and abdominal lymphadenopathy, bilateral pleural effusions and bone marrow infiltration, in keeping with stage IVB disease.[ncbi.nlm.nih.gov]
Cough
  • In fact, this mediastinal mass often provokes bilateral pleural effusion and pericardial effusion, which, in turn, may cause dyspnea, cough, chest pain, attenuation of breath sounds and impaired resonance as assessed by means of percussion.[symptoma.com]
  • This type of lymphoma may present itself with symptoms of cough, breathing difficulty or swelling of the head and neck due to the tumor pressing on the windpipe or large veins above the heart.[danafarberbostonchildrens.org]
  • This results in a mass in the chest that leads to breathing problems and cough. Water may also accumulate within the lungs. The bone marrow is commonly involved. The brain and spinal cord may also be affected in a number of cases.[verywell.com]
Dyspnea
  • Advances in Hematologic Malignancies Issue 1, Spring 2013 Figure 1 A 41-year-old man presented with 4 weeks of progressive dysphagia, reflux, involuntary weight loss, night sweats, dyspnea on exertion, and an enlarging left neck mass.[dana-farber.org]
  • In fact, this mediastinal mass often provokes bilateral pleural effusion and pericardial effusion, which, in turn, may cause dyspnea, cough, chest pain, attenuation of breath sounds and impaired resonance as assessed by means of percussion.[symptoma.com]
  • […] study (5) no history of previous anti-cancer therapy except listed below ; a) biopsy or surgical resection b) emergency irradiation therapy within 7days c) prednisolone therapy within 7 days d) VCR or DNR therapy within 7 days in order to improve severe dyspnea[upload.umin.ac.jp]
Chest Pain
  • In fact, this mediastinal mass often provokes bilateral pleural effusion and pericardial effusion, which, in turn, may cause dyspnea, cough, chest pain, attenuation of breath sounds and impaired resonance as assessed by means of percussion.[symptoma.com]
  • In 50-75% of people a central inner chest lump (mediastinal mass) occurs which may cause difficulty breathing (dyspnoea) and chest pain. Other areas of the body that can be affected include the thymus gland, liver, spleen, skin, testes and brain.[leukaemia.org.au]
Long Arm
  • Cytogenetic studies revealed that HT-1 cells carry an inversion (inv) of the long arm of chromosome 14.[ncbi.nlm.nih.gov]
Ptosis
  • A 58-year-old woman presented with headaches, right-sided ptosis and cranial nerve III palsy. She subsequently developed polyuria, polydipsia, and hyperglycemia and was found to have hypopituitarism.[ncbi.nlm.nih.gov]
Night Sweats
  • We report the simultaneous occurrence of T-cell lymphoblastic lymphoma (T-LBL) and WT in an 81-year-old patient, who presented with fever, night sweats and enlargement of the right parotid gland.[ncbi.nlm.nih.gov]
  • Advances in Hematologic Malignancies Issue 1, Spring 2013 Figure 1 A 41-year-old man presented with 4 weeks of progressive dysphagia, reflux, involuntary weight loss, night sweats, dyspnea on exertion, and an enlarging left neck mass.[dana-farber.org]
  • Patients may additionally present constitutional symptoms like fatigue, fever, night sweats and weight loss. Diagnostic imaging is of major importance to evaluate which organs are compromised by the disease.[symptoma.com]
  • Other symptoms include fever, weight loss, fatigue, and/or night sweats. Non-Hodgkin lymphoma ( NHL ) is a large group of cancers derived from lymphocytes (white blood cells).[cibmtr.org]
  • Symptoms include swollen lymph nodes, fatigue, weight loss, and night sweats . Diagnosis and Staging of Lymphoblastic Lymphoma To accurately diagnose and stage this disease, doctors may use blood work and bone marrow or lymph node biopsies.[lymphomainfo.net]
Breast Mass
  • We report on a 41-year-old woman with T-cell lymphoblastic lymphoma who presented with multiple bilateral breast masses. The patient was treated with intensive chemotherapy and mediastinal and whole-brain irradiation.[ncbi.nlm.nih.gov]
Facial Edema
  • The latter comprises distension of neck veins and raised jugular venous pressure as well as facial edema. The second most common site of solid tumor development in T-LBL patients are cervical, supraclavicular, and axillary lymph nodes.[symptoma.com]
Facial Edema
  • The latter comprises distension of neck veins and raised jugular venous pressure as well as facial edema. The second most common site of solid tumor development in T-LBL patients are cervical, supraclavicular, and axillary lymph nodes.[symptoma.com]

Workup

Diagnostic imaging is of major importance to evaluate which organs are compromised by the disease. In this context, computed tomography scans are usually recommended to visualize the thoracic cavity; fluorodeoxyglucose positron-emission tomography is highly valuable for the detection of neoplastic lymphadenopathy, and magnetic resonance imaging is mainly used to examine the central nervous system. Histopathological analysis of biopsy samples taken from affected lymph nodes or solid tumors may be required to define the origin of tumor cells. Furthermore, bone marrow and cerebrospinal fluid specimens have to be analyzed to confirm or rule out bone marrow involvement, T-ALL, and central nervous system compromise. In order to assess the general condition of the patient and to evaluate organ function, laboratory analyses of blood samples should be performed.

Findings obtained by those means may be used for tumor staging according to St Jude's staging classification for childhood non-Hodgkin lymphoma (Murphy staging) or the Ann Arbor staging system in pediatric and adult patients, respectively [13] [14]. These staging systems consider the following:

  • Sites of tumor growth
  • Tumors in thoracic and/or abdominal cavity
  • Nodal or extranodal presentation
  • Multiple organ involvement
  • Involvement of the bone marrow and/or central nervous system
  • Resectability of solid tumors
Mediastinal Mass
  • A case of T-cell lymphoblastic lymphoma is described in which the patient presented with a characteristic mediastinal mass and lack of bone marrow involvement.[ncbi.nlm.nih.gov]
  • This report describes a case of post-transplantation lymphoma presenting with a mediastinal mass causing superior vena cava syndrome, tracheal obstruction, and pleural effusion.[ncbi.nlm.nih.gov]
  • Abstract Typical adult cases of malignant lymphoma, diffuse, lymphoblastic type (MLLB), are of the T-cell immunophenotype and occur as mediastinal masses.[ncbi.nlm.nih.gov]
  • At the time of diagnosis, all patients had lymphadenopathy, and one had a mediastinal mass. Lymph node biopsies revealed lymphoblastic lymphoma admixed with a variable number of mature eosinophils.[ncbi.nlm.nih.gov]
  • Abstract A 10-year-old girl was diagnosed with lymphoblastic lymphoma; staging evaluation revealed a large mediastinal mass and normal peripheral blood and bone marrow morphology.[ncbi.nlm.nih.gov]
Pericardial Effusion
  • In fact, this mediastinal mass often provokes bilateral pleural effusion and pericardial effusion, which, in turn, may cause dyspnea, cough, chest pain, attenuation of breath sounds and impaired resonance as assessed by means of percussion.[symptoma.com]
Pericardial Effusion
  • In fact, this mediastinal mass often provokes bilateral pleural effusion and pericardial effusion, which, in turn, may cause dyspnea, cough, chest pain, attenuation of breath sounds and impaired resonance as assessed by means of percussion.[symptoma.com]
Chlamydia Trachomatis
  • At presentation, the patient also had a life-threatening pneumonia due to Chlamydia trachomatis which responded to erythromycin and tetracycline.[ncbi.nlm.nih.gov]
Pleural Effusion
  • RESULTS: G-banding and spectral karyotyping of pleural effusion cells showed 47,XY,dup(1)(q21q32),t(7;9)(q34;q34), 20.[ncbi.nlm.nih.gov]
  • A 3-year-old girl presenting with a huge mediastinal tumor and massive pleural effusion, was diagnosed with stage III γδ T-cell lymphoblastic lymphoma (γδ T-LBL) by flow cytometry of effusion cells.[ncbi.nlm.nih.gov]
  • Immunologic studies of the surface phenotype of the malignant cells in a pleural effusion with monoclonal antibodies revealed the cells to be of thymic origin and distinguished them from the surface phenotypes seen in T-cell acute lymphoblastic leukemia[ncbi.nlm.nih.gov]
  • This report describes a case of post-transplantation lymphoma presenting with a mediastinal mass causing superior vena cava syndrome, tracheal obstruction, and pleural effusion.[ncbi.nlm.nih.gov]
  • Staging investigations revealed mediastinal and abdominal lymphadenopathy, bilateral pleural effusions and bone marrow infiltration, in keeping with stage IVB disease.[ncbi.nlm.nih.gov]

Treatment

Distinct protocols have been applied to treat T-LBL patients. Best results in case of childhood lymphoma have been achieved applying the protocols described by Reiter et al. [2]. In brief, pediatric patients were subjected to multi-agent chemotherapy adapted to the severity of the disease (stage I/II or stage III/IV), and time passed since the initiation of treatment:

  • Induction, nine weeks of cyclophosphamide, daunorubicin, vincristine, prednisone plus L-asparaginase, cytarabine, mercaptopurine, methotrexate
  • Extracompartment-protocol M comprising the application of mercaptopurine and methotrexate for eight weeks
  • Re-induction in case of stage III/IV disease, consisting of cyclophosphamide, hydroxydaunorubicin, vincristine, dexamethasone plus L-asparaginase, cytarabine, thioguanine, methotrexate for seven weeks
  • Cranial radiation therapy in case of stage III/IV disease, due to relatively high risks of spread to the central nervous system, an unfavorable prognostic factor [10]
  • Maintenance therapy with mercaptopurine and methotrexate up to a total treatment period of two years

For adults diagnosed with T-LBL, similar protocols have been described [1]. Moreover, hematopoietic stem cell transplantation may be carried out after induction [3] [12].

While a determined regimen may be chosen upon diagnosis and staging, modifications may become necessary depending on the patient's response to therapy. Percentage of tumor regression, properties of residual tumors and resectability are primarily considered to this end. Available data indicate intense induction and consolidation to be crucial for success.

Prognosis

T-LBL is an aggressive neoplasm. For a long time, it has been associated with a poor outcome, but recently, event-free survival rates of up to 90% have been reported for pediatric patients [2]. In adults, such results have not yet been achieved, but there is an encouraging trend towards a better outcome. Recently, five- and seven-year overall survival rates of 42 and 51%, respectively, have been documented for adult patients [1] [3]. Unfortunately, the chance of survival drops drastically upon a relapse, e.g., to about 10% in case of childhood T-LBL [11]. Furthermore, female gender has been related to shorter survival, and it is tempting to speculate that this observation results from female patients being significantly older than men diagnosed with T-LBL [3]. Selected adult patients may benefit from hematopoietic stem cell transplantation [12].

Etiology

Malignant transformation of T cell precursors accounts for the development of T-LBL, and it has been speculated that such events may be triggered by exposure to radiation, by pesticides, congenital or acquired immunosuppression [5].

Chromosomal translocations involving promoters and enhancers of genes encoding for T cell receptor chains are frequently observed. Consequently, transcription factors like CDKN2A/2B, FBXW7, HOX11/TLX1 (homeobox 11/T-cell leukemia homebox 1), HOX11L2/TLX3 (homeobox 11-like 2/T-cell leukemia homebox 3), LYL1 (lymphoblastic leukemia-derived sequence 1), NOTCH1, and TAL1/SCL (T-cell acute lymphocytic leukemia 1/stem cell leukemia) are put under the control of those regulatory sequences. These chromosomal aberrations are associated with dysfunctional regulation of cell growth, division and differentiation. Interestingly, the precise mutations underlying an individual case of T-LBL seem to correlate with the outcome. For instance, NOTCH/FBXW7 are encountered in about half of T-LBL cases and are related to a good prognosis [6]. About a fourth of T-LBL patients shows chromosomal anomalies involving HOX11L2/TLX3, which may predict a worse outcome [7].

Epidemiology

T-LBL shows a bimodal age distribution, with higher incidence rates in people younger than 20 years and older than 50 years. With regards to pediatric patients, about one in three cases of non-Hodgkin lymphoma corresponds to T-LBL. This tendency is not reflected in the overall population, where T-LBL is considered a rare type of non-Hodgkin lymphoma that accounts for only 2% of cases. The overall incidence of lymphoblastic lymphoma has been estimated to 0.1-0.2 per 100,000 person-years, with the vast majority of those cases corresponding to T-LBL [8].

Males are affected more often than women. While the median age of T-LBL patients has been reported to be 40 years, females diagnosed with this lymphoproliferative disease are significantly older than males (e.g., 66 vs. 37 years as observed in Sweden [3]). Although racial predilection has been reported for other lymphoproliferative diseases, no significant differences have been encountered regarding the T-LBL incidence in African, Asian and Caucasian inhabitants of the United States [9]. In contrast, T-LBL incidence has been reported to correlate with socioeconomic development.

Sex distribution
Age distribution

Pathophysiology

T-LBL is characterized by an uncontrolled proliferation of lymphoblasts, whose belonging to the T cell lineage may be demonstrated by immunophenotyping. Immunohistochemical staining or flow cytometry may be applied to this end, with biopsy samples, pleuropericardic effusion specimens or other body fluids being best suited for testing [10]. Tumor cells obtained from T-LBL patients generally test positive for CD1a (cortical T cells) or CD3 (non-cortical T cells), whereas CD2 expression is frequently lost. CD4 and CD8 are not specific for T cells, but are not expressed by B cells. Staining for CD10 and CD34 may yield positive results in both T cell and B cell lymphoma. In few cases, mixed immunophenotypes and expression of markers of at least two distinct hematopoietic lineages may be encountered. In sum, T-LBL are highly heterogenous, tumor cells differ in chromosomal aberrations and maturity, and there is no one panel of markers expressed in any case. While the determination of the immunophenotype is of major importance for the diagnosis and classification of lymphoma, it has not yet been possible to relate the respective findings with the patient's response to therapy and outcome [2].

Prevention

No specific measures can be recommended to prevent T-LBL.

Summary

T-cell lymphoblastic lymphoma (T-LBL), previously designated precursor T-cell lymphoblastic lymphoma and sometimes also referred to as T-lymphoblastic lymphoma, is a rare lymphoproliferative disorder. According to the current classification of tumors of hematopoietic and lymphoid tissues, as published by the World Health Organization, T-LBL is a type of non-Hodgkin lymphoma associated with solid tumors in lymphatic tissues, hematological alterations and bone marrow involvement. In this context, T-LBL may be distinguished from T-cell acute lymphoblastic leukemia (T-ALL), with the two diseases characterized by bone marrow involvement below and above 25%, respectively [1]. Furthermore, they differ with regards to predominant lymphocytic phenotypes and gene expression profiles. In T-LBL, cortical and mature T cells prevail, whereas immature lymphocytes are more frequently encountered in T-ALL patients. There is no consensus whether these differences do indeed justify the definition of two malignant neoplasms, or whether they account for distinct forms of a single entity.

In any case, therapeutic regimens for T-LBL have almost exclusively been derived from protocols established for the treatment of T-ALL. They comprise CHOP chemotherapy, i.e., administration of cyclophosphamide, hydroxydaunorubicin, oncovin (vincristine), prednisone or prednisolone; CHOEP chemotherapy, which includes the application of etoposide; and hematopoietic stem cell transplantation. With regards to the success of those therapies, survival rates reported in literature speak for themselves: Intensive T-ALL therapy has been shown to be an effective treatment option for childhood T-LBL, and a five-year event-free survival rate of 90% has been achieved [2]. In a retrospective study recently conducted in Sweden, five-year overall survival in adults diagnosed with T-LBL has been observed to be 42% [3]. In contrast, in the 1980s, T-LBL has been described as an aggressive neoplasm with a median survival of about one year [4].

Patient Information

T-cell lymphoblastic lymphoma (T-LBL) is a rare lymphoproliferative disorder. It is triggered by chromosomal aberrations that lead to dysfunctional regulation of T cell growth, division and differentiation, but to date, little is known about the causes of the respective translocations of DNA sequence segments.

T cells or T lymphocytes are immune cells that fulfill a myriad of functions: They are able to recognize degenerated and foreign cells and may mediate their destruction, they may induce an immune response involving antibody-producing B lymphocytes, and they may also suppress such an immune response when the threat has past. Early T cell precursors may be encountered in the bone marrow, but these cells mature in an immune organ called thymus. The thymus is located in the mediastinum, close to the heart in the thoracic cavity.

The aforementioned chromosomal translocations generally occur in T cells undergoing maturation processes in the thymus. Because they proliferate in an uncontrolled manner, a solid mediastinal tumor may form, and this is indeed the most frequent finding in T-LBL patients. However, T cells may reach any tissue of the human body, and tumors may also grow in lymph nodes, liver, spleen and other organs. If tumor cells infiltrate the central nervous system or the bone marrow, the patient's prognosis worsens significantly.

Most patients are adolescents or adults aged 50 years and older. Upon diagnosis of T-LBL - which is a long process involving diagnostic imaging, histopathological analysis of biopsy specimens and analysis of bone marrow and cerebrospinal fluid - an appropriate treatment regimen is chosen. Most patients have to undergo long-term multi-agent chemotherapy, but protocols have repeatedly been adjusted to the needs of T-LBL patients and today, five-year-survival rates of 90% and 50% in pediatric and adult patients, respectively, may be achieved.

References

Article

  1. Hoelzer D, Gokbuget N. T-cell lymphoblastic lymphoma and T-cell acute lymphoblastic leukemia: a separate entity? Clin Lymphoma Myeloma. 2009; 9 Suppl 3:S214-221.
  2. Reiter A, Schrappe M, Ludwig WD, et al. Intensive ALL-type therapy without local radiotherapy provides a 90% event-free survival for children with T-cell lymphoblastic lymphoma: a BFM group report. Blood. 2000; 95(2):416-421.
  3. Ellin F, Jerkeman M, Hagberg H, Relander T. Treatment outcome in T-cell lymphoblastic lymphoma in adults - a population-based study from the Swedish Lymphoma Registry. Acta Oncol. 2014; 53(7):927-934.
  4. Streuli RA, Kaneko Y, Variakojis D, Kinnealey A, Golomb HM, Rowley JD. Lymphoblastic lymphoma in adults. Cancer. 1981; 47(10):2510-2516.
  5. Bach Okholm-Hansen A, Brorson S. Unexpected finding of T-cell lymphoma in a previously healthy 16-year-old patient after a thorax trauma: a case report. J Med Case Rep. 2014; 8:371.
  6. Park MJ, Taki T, Oda M, et al. FBXW7 and NOTCH1 mutations in childhood T cell acute lymphoblastic leukaemia and T cell non-Hodgkin lymphoma. Br J Haematol. 2009; 145(2):198-206.
  7. Ballerini P, Blaise A, Busson-Le Coniat M, et al. HOX11L2 expression defines a clinical subtype of pediatric T-ALL associated with poor prognosis. Blood. 2002; 100(3):991-997.
  8. Groves FD, Linet MS, Travis LB, Devesa SS. Cancer surveillance series: non-Hodgkin's lymphoma incidence by histologic subtype in the United States from 1978 through 1995. J Natl Cancer Inst. 2000; 92(15):1240-1251.
  9. Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. Blood. 2006; 107(1):265-276.
  10. Bassan R, Maino E, Cortelazzo S. Lymphoblastic lymphoma: an updated review on biology, diagnosis, and treatment. Eur J Haematol. 2016; 96(5):447-460.
  11. Bonn BR, Rohde M, Zimmermann M, et al. Incidence and prognostic relevance of genetic variations in T-cell lymphoblastic lymphoma in childhood and adolescence. Blood. 2013; 121(16):3153-3160.
  12. Song KW, Barnett MJ, Gascoyne RD, et al. Primary therapy for adults with T-cell lymphoblastic lymphoma with hematopoietic stem-cell transplantation results in favorable outcomes. Ann Oncol. 2007; 18(3):535-540.
  13. Murphy SB, Fairclough DL, Hutchison RE, Berard CW. Non-Hodgkin's lymphomas of childhood: an analysis of the histology, staging, and response to treatment of 338 cases at a single institution. J Clin Oncol. 1989; 7(2):186-193.
  14. Carbone PP, Kaplan HS, Musshoff K, Smithers DW, Tubiana M. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res. 1971; 31(11):1860-1861.

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Last updated: 2019-07-11 20:14