Human T cell lymphotropic virus (HTLV-1): cutaneous manifestations

Skin manifestations are a common feature of HTLV-1 associated disorders and of HTLV-1 infection itself. These include the lymphomatous skin infiltrates in adult T-cell lymphoma/leukaemia, most commonly manifesting as persistent, generalised papules, nodules and plaques with later ulceration, acquired ichthyosis and xeroderma in HAM/TSP, infective dermatitis of children, dermatomyositis, crusted (Norwegian) scabies, psoriasiform rashes which may precede one of the more serious disease associations, and possibly also seborrhoeic dermatitis. Disorders typically associated with immunosuppression such as disseminated herpes zoster, and ulcerative non-healing herpes simplex may also be seen occasionally both in ATK as well as in other wise asymptomatic HTLV-1 infection (AU)

Peripheral T cell lymphomas in Jamaica: relationship to HTLV-1 serostatus (abstract)

The clinico-pathologic features of 111 patients with non-Hodgkin's Lymphoma (NHL) exhibiting morphologic and immunophenotypic features of peripheral T-cell lymphoma (PTL) were analysed with respect to HTLV-I serostatus. 73 (65.8 percent) were HTLV-I seropositive (HTLV-I+) and 38 (34.2 percent) were HTLV-I seronegative (HTLV-I-). HTLV+ cases had a lower mean age (42.2 yrs) than HTLV- cases (50 yrs) and a male to female ratio of 1:1 while HTLV- cases showed a male preponderance (M:F 2:1). No significant differences in morphology were noted using the Japan Study Group classification. Both groups shared clinical features consistent with Adult T-cell leukemia/lymphoma but hypercalcemia was more likely to occur in the HTLV+ group [Hypercalcemia:- HTLV+ 50.7 percent, HTLV- 15.8 percent; Leukemia:- HTLV+ 39.7 percent, HTLV- 34.2 percent; Skin infiltration:- HTLV+ 38.3 percent, HTLV- 42.1 percent]. It would appear that some cases of HTLV- PTL could be included in the group classified as ATL given the occurrence of sentinel clinical features associated with ATL. Perhaps these will prove to be HTLV-I proviral DNA positive. Further studies to define this group are indicated. (AU)

Sibling adult T-cell leukemia/lymphoma and clustering of human T-Cell lymphotropic virus type I infection in a Jamaican family

BACKGROUND:- Human T-cell lymphotropic virus type I (HTLV-I) infection is endemic in Jamaica, with an estimated crude seroprevalence of 5percent. Adult T-cell lymphoma/Leukemia (ATL), a disease caused by HTLV-I, has an incidence of 1-2/100,000 in the Jamiacan population. Familial ATL has not previously been reported from Jamaica. METHODS:- Hospital records and histologic specimens of the two cases were reviewed. HTLV-I infection was confirmed by antibody testing and by polymerase chain reaction on paraffin-embedded tissue,where serum was unavailable. Family members identified by the patient's parents. After giving informed consent, family members were asked to complete an interviewer-administered questionnaire and to agree to phlebotomy. RESULTS:- ATL developed 10 years apart in two siblings from a Jamaican family at age 16 and 24 years. A study of 19 members of their extended family, including both parents, 2 grandparents, and 3 siblings, revealed an overall HTLV-I seroprevalence of 17 percent. This compared with 75 percent among parents and sibling living in the same household as the patients (AU)

Fatal metastatic calcification in a patient with HTLV-I-associated lymphoma

Metastic calcification of the heart, lungs and kidneys is described in a 42-year-old male who had an HTLV-I-associated lymphoma. This fatal complication of HTLV-I-associated lymphoma has been infrequently reported, and the case in question is the first to be recorded in the Caribbean, where HTLV-I is common. Aggressive therapy of hypercalcaemia should be instituted early in its genesis in these lymphomas, so as to avoid its fatal outcome (AU)

Molecular epidemiology of HTLV-I-associated non-Hodgkin's lymphomas in Jamaica

As part of epidemiologic studies of human T-lymphotropic virus (HTLV)-I-associated malignancies in Jamaica, the authors evaluated 26 patients with non-Hodgkin's lymphoma for the presence of integrated HTLV-I provirus in their malignant cells. Fifteen of 26 patients had integrated provirus. All 15 also were HTLV-I antibody positive. Eleven patients did not have integrated provirus, and all 11 were antibody negative. All of the antibody-positive cases had onset of their disease in adulthood (age range, 21-57 years) as opposed to the broad age range of negative cases (4-66 years). Clinical features which were more common in provirus positive than negative patients included leukemic phase, skin involvement, and hypercalcemia, which are all features frequently seen in HTLV-I-associated adult T-cell leukemia/lymphoma (ATLL). The presence of skin involvement, circulating malignant cells, abnormal liver function tests, or the presence of two or more of these four features were statistically significantly different between virus-positive and virus-negative cases. Although the survival of positive cases (6 months) was shorter than that of negative cases (9 months), this was not statistically significant. The only significant determinant of survival was hypercalcemia, with those who developed hypercalcemia at some point in their disease course, independent of their HTLV-I status, surviving a mean of 5 months as compared to a mean of 17.5 months in those who never became hypercalcemic. The six HTLV-I-positive lymphomas that underwent cell typing were all primarily OKT4 positive, whereas two HTLV-I antibody-negative cases that were typed were B-cell lymphomas. (AU)

Non-hodgkin lymphoma in Jamaica and its relation to adult T-cell leukemia-lymphoma [published erratum appears in Ann Intern Med 1987 Jun;106(6):917]

Of 95 patients consecutively diagnosed with non-hodgkin lymphoma, 52(55 percent) had antibodies to human T-cell leukemia-lymphoma virus, type I. Antibody positively was strongly associated with skin involvement, leukemia, and hypercalcemia (p<0.02). Two patients had systemic opportunistic infections. Neither meningeal nor lung infiltration was detected, and lymph node infiltration was diffuse in all patients. Of 36 patients who received immunophenotypic classifications, 30 had diseases that affected the T-cell system, and the cells of all tested patients with these diseases showed the helper/inducer (T4) phenotype. Twenty-seven of these thirty-six patients were found to have adult T-cell leukemia-lymphoma, and of the 27, 24 had antibodies to HTLV-I. The median duration of survival in patients with adult T-cell leukemia-lymphoma was 17 weeks, but a subgroup of 81 weeks, which suggests that the disease has differing expression with courses that range from smoldering and indolent to acute and rapidly fatal. Hypercalcemia was the most important prognostic determinnant of adult T-cell leukemia-lymphoma.(AU)

The aetiology of hypercalcaemia at the University Hospital of the West Indies (1978-1983)

A retrospective study of the aetiology of hypercalcaemia at the University Hospital of the West Indies over the six-year period 1978-1983 was undertaken. In contrast to other studies where solid malignant tumours, with or without bony metastases, were the commonest cause of hypercalcaemia, it was found that haematological malignancies were the commonest identifiable causes. This was followed by chronic renal failure, primary hyperparathyroidism and solid malignant tumours in that order. The reason for this difference is more likely a reflection of the avidity with which hypercalcaemia is sought by the different clinicians. In the majority of cases of hypercalcaemia, the aetiology was not ascertained either due to inadequate investigation or because the patients were lost to follow-up. Interestingly, in this category, about one quarter of the patients were found to have renal stones (AU)

Simple biochemical tests to distinguish primary hyperparathyroidism from other causes of hypercalcaemia

Calcium, the most common divalent cation in the body, is a frequently requested test. Disorders of calcium metabolism result in either hypocalcaemia or more commonly hypercalcaemia. Hypercalcaemia occurs usually in association with malignant disease or hyperparathyroidism. The diagnosis of primary hyperparatyroidism is sometimes difficult especially if parathormone levels are not available. Some routine biochemical tests - serum chloride, bicarbonate, calcium, phosphate and alkaline phosphatase levels and the chloride/phosphate ratio - were examined to determine how useful they were in distinguishing primary hyperparathyroidism from other causes of hypercalcaemia. All cases with a serum creatinine level over 130 umol/l were excluded. The chloride/phosphate ratio and chloride and phosphate levels were the most useful. The bicarbonate concentrations showed no significant differences. Serum calcium and alkaline phosphatase levels were also found to be useful although these values tend to be lower in patients whose hypercalcaemia was caused by diseases other than malignancy or hyperparathyroidism (AU)

Adult T-cell leukemia/lymphoma in Jamaica and its relationship to human T-cell leukemia/lymphoma virus type I-associated lymphoproliferative disease

We had shown previously that the prevalence of human T-cell leukemia/lymphoma virus type I (HTLV-I) antibody positivity is high in Jamaican non-Hodgkin's lymphoma (NHL) patients and that virus-positive patients have the clinical features and poor prognosis of adult T-cell leukemia/lymphoma (ATL). 62 percent of 45 NHL patients diagnosed consecutively between 2/1/82 and 1/31/84 and studied prospectively were HTLV-I-antibody positive. Skin involvement (38 percent), hypercalecemia (44 percent), and leukemia (40 percent) were unusually prevalent and there was a strong association (p < 0.05) with HTLV-I-antibody positivity. 52 percent of the patients had bone marrow infiltration, and 74 percent of these patients were HTLV-I-antibody positive (p=.06). Lymphadenopathy (96 percent), hepatomegaly (60 percent), and splenomegaly (25 percent) were detected with about the same frequency as in other series of NHL patients with advanced disease, and 61-88 percent of these patients were HTLV-I-antibody positive. Patients were classified into those with "typical ATL" (NHL associated with 2 of the 4 features) i) hypercalecemia; ii) histologically proven skin infiltration; iii) leukemia; and iv) bone marrow infiltration, providing that the morphology of infiltrating of leukemic cells was characteristic of ATL; those "consistent with ATL" (NHL associated with 1 of these 4 features); and "non-ATL" (NHL without any of these 4 additional features). Thirty-two (71 percent) of the NHL patients were ATL patients, i.e. had features typical of or consistent with ATL, and 78 percent of these were HTLV-I-antibody positive. HTLV-I provirus was detected in tumour cells of all HTLV-I-antibody positive patients tested. Three (23 percent) of the non-ATL patients were HTLV-I-antibody positive. There was no correlation between histopathological features and the clinical classification of HTLV-I-antibody positivity. Median survival of ATL and non-ATL patients was 16 and 53 weeks. Although the disease was unusually fulminant, 34 percent of the ATL patients had a subacute or chronic course. Skin involvement and leukemia were prominent in these patients. Hypercalecemia was the chief prognostic determinant. Median < 0.05). Hypercalecemia caused 10 deaths, infections 12, and death was due to tumour progression in 4 patients. Infections were usually due to pyogenic organisms and only 2 patients had systemic opportunistic infections. Six (27 percent) of 22 chronic lymphocytic leukemia (CLL) patients were HTLV-I-antibody positive. (AU)

Primary hyperparathyroidism at the U.H.W.I

Twenty-two cases of primary hyperparathyroidism were seen at the University Hospital of the West Indies during the twelve year period, January, 1966, to December 1977. Three groups of patients were identified depending on the main symptom complex at presentation: (1) renal calculi (2) bone pain and (3) symptoms of hypercalcaemia. All cases had elevated serum calcium levels. Twenty-one patients were found to have a single parathyroid adenoma at surgery and there was one case of hypersia. No cases with the multiple endocrine adenoma syndrome were identified. The pathology of the disease is described, aspects of diagnosis are outlined and methods of tumour localization are discussed (AU)

Hypercalcaemia with features resembling rickets in five female siblings

A study was made of an Indian family in Trinidad in which five sisters were similarly affected with an abnormality involving mainly the lower limbs and showing radiological features of rickets. The limb deformities became apparent between 2-4 years of age soon after they started to walk. Three brothers and a sister have not so far shown evidence of this abnormality. Four of the sisters have had raised serum calcium and alkaline phosphatase levels and have shown gradual improvement in the bony deformity without any special treatment (AU)