Adult T-cell leukemia overview

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Adult T-cell leukemia Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Adult T-cell leukemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-Ray Findings

Echocardiography and Ultrasound

CT scan

MRI Findings

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Case #1

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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[1][2].

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. [3]

Classification

Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an acute variant, chronic variant, smoldering variant, or an adult T-cell lymphoma variant.

Pathophysiology

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) [4]. 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.

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.

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.

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] [6].

Risk Factors

Common risk factors in the development of adult T-cell leukemia among HTLV carriers are vertical transmission of HTLV infection during labor, male sex, and specific human leukocyte antigens such as HLA-A 26, HLA-B 4002, and HLA-B 4801.

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.

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, whereas chronic and smouldering subtypes have a good prognosis.

Diagnosis

Diagnostic Study of Choice

There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic histopathology and HTLV-1 infection.

History and Symptoms

Symptoms of adult T-cell leukemia include fatigue, fever, night sweats, constipation, and recurrent infections.

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.

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.

Electrocardiogram

There are no ECG findings associated with adult T-cell leukemia. However, electrocardiogram might be helpful in detecting complications of adult T-cell leukemia such as cardiac arrhythmias due to hypercalcemia. To view the electrocardiogram findings in hypercalcemia, click here.

X-ray

There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia which include bone fractures and lytic lesions.

Echocardiography and Ultrasound

There are no echocardiography findings associated with adult T-cell leukemia. Findings on an ultrasound suggestive of adult T-cell leukemia include hepatomegaly, splenomegaly and lymphadenopathy.

CT Scan

Thoracic CT scan may be helpful in the diagnosis of adult T-cell leukemia. 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. Findings on abdominal CT scan suggestive of adult T-cell leukemia cells include hepatomegaly and splenomegaly.

MRI

There are no MRI findings associated with adult T-cell leukemia.

Other Imaging Findings

There are no other imaging findings associated with adult T-cell leukemia.

Other Diagnostic Studies

Other diagnostic studies for adult T-cell leukemia include skin biopsy, bone marrow biopsy, and fluorescent in situ hybridization.

Treatment

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.

Interventions

There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.

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.

Primary Prevention

Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of HTLV virus. No preventive vaccine against HTLV-1 is currently available.

Secondary Prevention

There are no established measures for the secondary prevention of Adult T-cell leukemia.

References

  1. Guru Murthy GS, Pondaiah SK, Abedin S, Atallah E (November 2018). "Incidence and survival of T-cell acute lymphoblastic leukemia in the United States". Leuk. Lymphoma: 1–8. PMID 30407885 . doi:10.1080/10428194.2018.1522442. 
  2. Fernández MS, Rojas FD, Cattana ME, Mussin JE, de Los Ángeles Sosa M, Benzoni CD, Giusiano GE (2017). "Protothecosis in a patient with T cell lymphocytic leukemia". Rev. Argent. Microbiol. 49 (3): 224–226. PMID 28554708. doi:10.1016/j.ram.2017.02.007. 
  3. "Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H. Adult T-cell leukemia: clinical and hematologic features of 16 cases. Blood. 1977;50(3):481-492". Blood. 128 (24): 2745. December 2016. PMID 27979862. doi:10.1182/blood-2016-10-746677. 
  4. Santos RF, Conceição GC, Martins MS, Kraychete A, Penalva MA, Carvalho EM, Lopes AA, Rocha PN (February 2017). "Prevalence and risk factors for Human T-Lymphotropic Virus Type 1 (HTLV-1) among maintenance hemodialysis patients". BMC Nephrol. 18 (1): 64. PMC 5312583Freely accessible. PMID 28202003. doi:10.1186/s12882-017-0484-y. 
  5. Kawano N, Yoshida S, Kawano S, Kuriyama T, Tahara Y, Toyofuku A, Manabe T, Doi A, Terasaka S, Yamashita K, Ueda Y, Ochiai H, Marutsuka K, Yamano Y, Shimoda K, Kikuchi I (September 2018). "The clinical impact of human T-lymphotrophic virus type 1 (HTLV-1) infection on the development of adult T-cell leukemia-lymphoma (ATL) or HTLV-1-associated myelopathy (HAM) / atypical HAM after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and renal transplantation". J Clin Exp Hematop. 58 (3): 107–121. PMID 30089749 . doi:10.3960/jslrt.18011. 
  6. Iwanaga M, Watanabe T, Yamaguchi K (2012). "Adult T-cell leukemia: a review of epidemiological evidence". Front Microbiol. 3: 322. PMC 3437524Freely accessible. PMID 22973265. doi:10.3389/fmicb.2012.00322. 

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