Human T-Cell Lymphotropic Viruses Clinical Presentation

Updated: Oct 05, 2015
  • Author: Ewa Maria Szczypinska, MD; Chief Editor: Pranatharthi Haran Chandrasekar, MBBS, MD  more...
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Presentation

History

Acute human T-cell lymphotropic virus (HTLV) infection is rarely suspected or diagnosed.

Because of the low viral replication of HTLV, there are usually no clinical symptoms. Diagnosis may stem from blood donation, testing performed because of a familial history of the infection, or a workup of adult T-cell leukemia (ATL) or HTLV-1–associated myelopathy/tropical spastic paraparesis (HAM/TSP) in patients with consistent clinical manifestations. Suspected cases may prompt investigation for a history of a recent blood-product transfusion or a nursing mother from an endemic area.

In considering HTLV infection, the most important historical information pertains to risk assessment. Because detecting accurate seroprevalence in low-endemic populations is inherently problematic, it is important to stratify a patient's risk. Screening enzyme immunoassays (EIAs) yield false-positive results in more than 50% of cases in areas of low prevalence. Therefore, a high-risk individual is anyone who has any of the following characteristics:

  • Has lived or lives in an endemic area (ie, Japan, the Caribbean, Central or West Africa, South America)
  • Is a Native American Indian
  • Has parents or sexual partners from an endemic area
  • Received blood-product transfusions in the United States before 1988
  • Has received blood transfusions anywhere that lacks active blood-bank screening
  • Has a history of injection drug use
  • Has sexual partners with a history of injection drug use
  • Has multiple sexual partners and does not use barrier protection
  • Has strongyloidiasis hyperinfection

The sequelae of latent HTLV infection generally occur decades after the initial infection, with the one exception being the reports of HAM/TSP occurring within a few months of HTLV-1–contaminated blood transfusion. [40]

Patients with HAM/TSP may present with weakness and stiffness in the lower limbs (first presenting symptom in 60% of cases [41] ), urinary incontinence, and/or severe lower back pain radiating to the legs. In some cases, urinary frequency, urgency, incontinence, or retention precedes the paraparesis by many years. Infected patients may also have symptoms of autonomic dysfunction leading to constipation, and, in some cases, sexual dysfunction.

Symptoms of ATL are clinically broad and can manifest as fatigue, overt lymphadenopathy, thirst (due to hypercalcemia), nausea, vomiting, fever, or abdominal pain.

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Physical

There are no strict criteria established in terms of physical findings of HAM/TSP; however, the following constellation of physical findings are typical and progressively worsen: [27]

  • Motor and sensory changes in the lower extremities
  • Clonus (may be evident); involuntary muscular contractions upon stretching of the muscles
  • Spastic gait in combination with weakness of the lower limbs
  • Detrusor insufficiency leading to bladder dysfunction
  • Preserved cognitive and upper-extremity neurological functions

The following isolated neurological symptoms have been also described in patients affected with HTLV-1 or HTLV-2: [27]

  • Sensory neuropathies
  • Gait abnormalities
  • Bladder dysfunction
  • Mild cognitive defects
  • Motor abnormalities

ATL has the following 4 distinct types and clinical characteristics:

  • Acute ATL
    • Short and aggressive clinical course
    • Hypercalcemia, lytic bone lesions, pulmonary involvement, and lymphocytosis
    • Hepatosplenomegaly
    • Cutaneous lesions (indolent, nodular, indurated, exfoliative, or erythrodermal)
  • Smoldering ATL
    • Abnormal lymphocytes of 5% or less
    • Malignant cells with monoclonal proviral integration
    • Skin lesions
    • Pulmonary involvement (occasional)
    • No hypercalcemia, lymphadenopathy, or other visceral involvement
    • Possible elevation of the serum lactase dehydrogenase level
  • Chronic ATL
    • No hypercalcemia, ascites, or pleural effusion
    • No CNS, bone, or GI involvement
    • Possible lymphadenopathy, hepatomegaly, splenomegaly, skin or pulmonary involvement
    • A serum lactate dehydrogenase level that may be twice the reference range
    • Abnormal T-cell lymphocytes, greater than 3.5 X 109/L
    • Absolute lymphocytosis, greater than 4.0 X 109/L
  • Lymphomatous ATL
    • Lymphadenopathy in the absence of lymphocytosis
    • Histologic evidence of lymph node involvement required
    • Skin lesions clinically indistinguishable from cutaneous T-cell lymphomas
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Causes

HTLV-1 and HTLV-2 are transmitted vertically from mother to child via breastfeeding or childbirth, from person to person through sexual contact, and through blood contact, either by transfusion or by reuse of injection equipment.

Blood transfusion is very effective at transmitting HTLV-1 and HTLV-2. Screening is standard policy in the United States and in many other countries. The United States has been screening donated blood since 1988.

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