Search

This case study explores a clinicopathological presentation of Adult T-cell leukaemia/lymphoma (ATLL) at Tygerberg Hospital; a disease associated with adulthood noted in an adolescent patient. Adult T-cell leukaemia–lymphoma oncogenesis develops through a multistep process with an accumulation of mutations. Infection through human T-lymphotropic virus type 1 (HTLV-1) is the first step of a multistep process resulting in eventual clonal proliferation of mature T-cells. There is a long latency period of 20–50 years from the time of infection with HTLV-1 to the development of symptoms of ATLL; thus, ATLL is a malignancy associated with adulthood. The median age of diagnosis is 58, ranging from the third to ninth decade of life. This is an ideal learning case as it highlights the importance of recognising ATLL in children and young adults in our population.

'Flower cells' are described typically in HTLV-1 induced adult T cell leukemia. Very few case reports have been published documenting flower cells in B cell lymphomas. We herein present a case of Burkitt lymphoma proven on the basis of immunophenotyping and FISH studies, with 'flower cells' morphology. [ABSTRACT FROM AUTHOR]

Abstract: Human T-cell lymphotropic virus type 1 (HTLV-1)-associated adult T-cell leukemia/lymphoma (ATLL) has a poor prognosis owing to its intrinsic resistance to chemotherapy. Although zidovudine (AZT) and alpha interferon (IFN-α) give rise to some response and improve the prognosis of ATLL, alternative therapies are needed. Arsenic trioxide (As2O3) has been shown to synergize with IFN-α in arresting cell growth and inducing apoptosis of ATLL cells in vitro. In this study, we evaluated the toxicity and the efficacy of this combined treatment in HTLV-1-infected squirrel monkeys (Saimiri sciureus) and HTLV-1 infected cell lines derived therefrom. We first show that treatment with As2O3 and IFN-α can induce growth arrest in HTLV-1-transformed monkey T-cell lines in vitro. We then show that treatment of squirrel monkeys with As2O3 in vivo is highly toxic at 0.9 or 0.3mg/day but not at 0.14mg/day for up to 2 weeks. Although the combination of As2O3 and IFN-α did not affect significantly the HTLV-1 proviral load in infected monkeys, it reduced the absolute numbers of CD3+, CD4+ and CD8+ cells during treatment, with a significant reduction in the total number of circulating HTLV-1 flower cells in the infected monkeys with chronic ATLL-like disease. [Copyright &y& Elsevier]

Large lymphocytes with basophilic cytoplasm and cleaved/cerebriform nuclei called flower cells have been described in human T-lymphotrophic virus type I (HTLV-I) seropositive individuals and may be precursors of adult T-cell leukaemia (ATL). A cohort of 546 HTLV-seropositive former blood donors, 32 HTLV-positive sexual partners of these donors and 799 HTLV-seronegative controls has been followed as part of the Retrovirus Epidemiology Donor Study. A novel methodology was developed to systematically review peripheral blood slides from these subjects for HTLV-related lymphocyte abnormalities, using an algorithm based on morphologic features to objectively identify flower cells. The algorithm included: absence of azurophil granules; nuclear chromatin condensation; cell size >1.5 small lymphocytes; nuclear to cytoplasmic ratio >80%; and presence of nuclear folding/lobulation. Peripheral slides from subjects were screened by a medical technologist blinded to HTLV status. 6.8% of HTLV-I subjects (P = 0.0001 versus seronegatives), 0.9% of HTLV-II subjects and 1.1% of seronegatives were confirmed to have cells classified as flower cells by two haematologists using objective criteria, and blinded to serostatus. Despite the higher prevalence of flower cells in HTLV-I positives, no clinical correlations were found. Longitudinal follow-up may yield higher rates of cellular abnormalities as the sequelae of HTLV infection develop. [ABSTRACT FROM AUTHOR]

Abstract: Background: Infective dermatitis associated with HTLV-1 (IDH) is a severe childhood form of eczema that may progress to adult T-cell leukemia/lymphoma (ATL). Objective: In this study, the presence of clinical and laboratory parameters suggestive of ATL was evaluated in a cohort of 30 patients with IDH. Study design: Over a period of 33 months, the patients were submitted to three-monthly clinical evaluations, routine laboratory exams, full blood count and blood smears, and to six-monthly blood sampling for HTLV-1 proviral load determination. HTLV-1 proviral load was quantified using real-time TaqMan PCR assay. Results: Abnormal cells (Ably) were found in the peripheral blood smears of nine patients (30%), flower cells being detected in five of these cases (16.6%). The presence of Ably and flower cells was not associated with a higher proviral load in those patients. Conclusions: This is the first report on the presence of flower cells in HTLV-1-infected children and adolescents. Furthermore, these cells have not previously been reported in IDH patients. The cases with flower cells probably represent precursory ATL cases, these patients being at a greater risk of developing ATL. [Copyright &y& Elsevier]