Correspondence

Adult T-Cell Leukemia–Lymphoma during Pregnancy

N Engl J Med 2002; 346:2014-2015June 20, 2002

Article

To the Editor:

Human T-cell lymphotrophic virus type I (HTLV-I) infection is uncommon in the United States. During the period from 1991 to 1996, the incidence of seropositivity for HTLV-I or human T-cell lymphotrophic virus type II (HTLV-II) in U.S. blood donors was 1.59 per 100,000 person-years.1 This virus causes adult T-cell leukemia–lymphoma.2 We report a case of adult T-cell leukemia–lymphoma during pregnancy.

A 23-year-old woman was admitted to a hospital in South Carolina because of a 7-day history of sore throat, fever, and fatigue during the 26th week of gestation. There was no history of intravenous drug use, foreign travel, transfusion, or sexual promiscuity. Exudative tonsillitis and tender, enlarged lymph nodes of the head and neck were present. An enlarged spleen (20 cm) and stable fetal status were confirmed on ultrasonography. The white-cell count was 55,900 per cubic millimeter (with 74 percent unclassified cells). Serum aminotransferase levels were normal; a Monospot test, a test for antistreptolysin O titers, a polymerase-chain-reaction (PCR) assay for human immunodeficiency virus type 1, and tests for IgM antibodies against Epstein–Barr virus, human cytomegalovirus, and Toxoplasma gondii were negative. The patient was given supportive care.

On the fourth hospital day, the white-cell count rose to 75,000 per cubic millimeter, the serum calcium level was 20.2 mg per deciliter, and the lactate dehydrogenase level was 7056 U per liter. Flow-cytometric analysis showed increased numbers of CD3+CD4+ and CD3–CD25+ T cells in the blood, and examination of a specimen from a bone marrow biopsy showed 34 percent atypical, intermediate-size lymphocytes. IgG anti–HTLV-I or HTLV-II antibodies were present, and HTLV-I was detected by a PCR assay performed on the whole-blood sample. The patient was treated with hydroxyurea (1 g) and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy; the white-cell count fell to 12,700 per cubic millimeter. On the eighth day, a healthy boy was delivered by cesarean section. Eleven days after admission, the patient died from Staphylococcus aureus septicemia.

HTLV-I is endemic on the islands of the Caribbean basin and in Japan, South America, and West and Central Africa. In the United States, most cases of HTLV-I or HTLV-II infection are reported on the East and West Coasts.3 We do not know how this patient became infected with HTLV-I.

In the United States, the rate of vertical transmission of HTLV-I from infected mothers to offspring is approximately 2.7 percent. Adult T-cell leukemia–lymphoma develops in approximately 5 of 100 patients with chronic HTLV-I infection, and the risk of adult T-cell leukemia–lymphoma among children born to women who are seropositive for HTLV-I ranges from 0.1 percent to 1 percent.4 Breast-feeding is the main route of transmission, and prolonged exposure to breast milk (>3 months) increases the rate of postpartum transmission of HTLV-I by approximately 20 percent.5 Prenatal HTLV-I screening of high-risk women in the coastal regions of the United States may provide critical information, since the cessation of breast-feeding by HTLV-I–infected women may prevent retroviral transmission to the child.

Amar Safdar, M.B., B.S., M.D.
Natalie Johnson, M.D.
Francisco Gonzalez, M.D.
John D. Busowski, M.D.
University of South Carolina School of Medicine, Columbia, SC 29203-6876
safdar@richmed.medparksc.edu

5 References

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   Takezaki T, Tajima K, Ito M, et al. Short-term breast-feeding may reduce the risk of vertical transmission of HTLV-I. Leukemia 1997;11:Suppl 3:60-62
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Citing Articles (1)

Citing Articles

1. 1

   Mignon L. Loh, Katherine K. Matthay. 2005. Congenital Malignant Disorders. , 1437-1470.
   CrossRef