Adult T-cell leukemia overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune response. Development of adult T-cell leukemia is the result of multiple genetic mutations induced by an infection with human T‐cell lymphotropic virus (HTLV). On gross pathology, skin nodules, maculopapular eruption, and erythema are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated nucleus and agranular cytoplasm. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an acute variant, chronic variant, smoldering variant, or an adult T-cell lymphoma variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include cardiac arrhythmias, opportunistic infections , and bone fractures. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, whereas chronic and smoldering subtypes have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy. Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.
Historical Perspective
Adult T-cell leukemia was first discovered by Dr. K. Takatsuki, a Japanese physician, in 1977. The association between HTLV infection and adult T-cell leukemia was made in 1981.[1][2]
Classification
Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an acute variant, chronic variant, smouldering variant, or a lymphoma variant.[3][4]
Pathophysiology
Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune response.[3][5][6][7][8] Development of adult T-cell leukemia is the result of multiple genetic mutations induced by an infection with human T‐cell lymphotropic virus (HTLV). On gross pathology, skin nodules, maculopapular eruption, and erythema are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobated nucleus and agranular cytoplasm.
Causes
Adult T-cell leukemia is caused by an infection with HTLV. Common genetic mutations involved in the development of adult T-cell leukemia can be found here.[3][5][6]
Differentiating Adult T-cell leukemia from other Diseases
Adult T-cell leukemia must be differentiated from other diseases that cause weight loss, night sweats, hepatosplenomegaly, and palpable lymph nodes, such as hairy cell leukaemia, prolymphocytic leukaemia, follicular lymphoma, and mantle cell lymphoma.[5][9]
Epidemiology and Demographics
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted incidence rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age; the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia.[5][1][10][11]
Risk Factors
Common risk factors in the development of adult T-cell leukemia among HTLV carriers are vertical transmission of HTLV infection during infancy, male sex, and specific human leukocyte antigens such as HLA-A 26, HLA-B 4002, and HLA-B 4801.[11]
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for adult T-cell leukemia.[12]
Natural History, Complications and Prognosis
The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include cardiac arrhythmias, opportunistic infections , and bone fractures. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good prognosis.[3][5][13][1]
Diagnosis
History and Symptoms
Symptoms of adult T-cell leukemia include fatigue, fever, night sweats, constipation, and reccurrent infections.[3][5][1]
Physical Examination
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for maculopapular rash, skin ulceration, and splenomegaly.[3][5]
Laboratory Findings
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal anemia, thrombocytopenia, and elevated lymphocyte count. Hypercalcemia is a key feature among patients with adult T-cell leukemia.[3][4][1]
CT Scan
Thoracic CT scan may be helpful in the diagnosis of adult T-cell leukemia.[14] Findings on CT scan suggestive of pulmonary infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral lung parenchyma, and ground-glass attenuations.
Other Diagnostic Studies
Other diagnostic studies for adult T-cell leukemia include skin biopsy, bone marrow biopsy, and fluorescent in situ hybridization.[3][15]
Medical Therapy
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy. Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.[16]
Surgery
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. Splenectomy is usually reserved for certain cases of adult T-cell leukemia.[15]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 Mahieux R, Gessain A (2007). "Adult T-cell leukemia/lymphoma and HTLV-1". Curr Hematol Malig Rep. 2 (4): 257–64. doi:10.1007/s11899-007-0035-x. PMID 20425378.
- ↑ Tsukasaki K (2012). "Adult T-cell leukemia-lymphoma". Hematology. 17 Suppl 1: S32–5. doi:10.1179/102453312X13336169155330. PMID 22507774.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Matutes E (2007). "Adult T-cell leukaemia/lymphoma". J. Clin. Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
- ↑ 4.0 4.1 Shimoyama M (1991). "Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87)". Br J Haematol. 79 (3): 428–37. PMID 1751370.
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 5.6 Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015
- ↑ 6.0 6.1 Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015
- ↑ Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015
- ↑ Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015
- ↑ Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011
- ↑ Satake M, Yamada Y, Atogami S, Yamaguchi K (2015). "The incidence of adult T-cell leukemia/lymphoma among human T-lymphotropic virus type 1 carriers in Japan". Leuk Lymphoma. 56 (6): 1806–12. doi:10.3109/10428194.2014.964700. PMID 25219595.
- ↑ 11.0 11.1 Iwanaga M, Watanabe T, Yamaguchi K (2012). "Adult T-cell leukemia: a review of epidemiological evidence". Front Microbiol. 3: 322. doi:10.3389/fmicb.2012.00322. PMC 3437524. PMID 22973265.
- ↑ Recommendations. US Preventive Service (2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Adult+T-cell+leukemia+ Accessed on January, 24 2015
- ↑ Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y; et al. (2015). "Treatment and survival among 1594 patients with ATL". Blood. 126 (24): 2570–7. doi:10.1182/blood-2015-03-632489. PMID 26361794.
- ↑ Okada F, Ando Y, Kondo Y, Matsumoto S, Maeda T, Mori H (2004). "Thoracic CT findings of adult T-cell leukemia or lymphoma". AJR Am J Roentgenol. 182 (3): 761–7. doi:10.2214/ajr.182.3.1820761. PMID 14975983.
- ↑ 15.0 15.1 Leukemia - Chronic T-Cell Lymphocytic: Diagnosis. Cancer.Net (2015) http://www.cancer.net/cancer-types/leukemia-chronic-t-cell-lymphocytic/diagnosis Accessed on January, 25 2016
- ↑ Adult T-Cell Leukemia/Lymphoma. NCCN Guidelines Version 2 (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on January, 25 2016