Adult T-cell leukaemia or lymphoma is a definite peripheral T-lymphocytic malignancy related with a retrovirus designated by the human T-cell lymphotrophic virus type 1(HTLV-1).An exposure to the HTLV-1 at early years of life is one of the predisposing factors, which lead to the development of ATLL. The disease explicit with leukaemia in greater than two thirds of patients meanwhile other patients have lyphomatous form. Acute lymphoma, chronic lymphoma and smouldering are the clinicopathologic courses that have been identified and they result in the manifestation of adult t-cell leukaemia that have different forms which vary in prognosis. Most common characteristics of the disease are hypercalcemia, organomegaly and circulating atypical lymphocytes (flower cells) with CD4+ CD25+ phenotype. Furthermore, in HTLV-1 carries the progressive incidence is estimated to be 2.5%. Adult t-cell diagnosis is established of clinical features and laboratory investigations. Moreover, the final consists of lymphocyte morphology and immunophenotype of the affected tissues in the pure lymphoma forms and DNA analysis for HTLV-1. The diagnosis of the adult t-cell lymphoma should be based on a sequence of clinical features and laboratory investigations. ATLL and HTLV-1 are extensive in Japan, the Caribbean basin, and certain regions of South America and Africa. The disease is occasionally circulated, despite of the lymphomas that have been recognised in the gut and central nervous system. The perspective of the ATLL is poor as most patients do not respond nor have impermanent partial responses to treatments used for other T-cell lymphomas. The article will focus on clinical features, laboratory diagnostic features such as looking into the peripheral blood, bone marrow and lymph nodes.
ATLL affects adults only and is remarkably rare in children, despite the fact that a couple of cases in childhood have been reported. The middle age is around the mid?60s and there is no gender pervasiveness. In Japan, USA and England familial ATLL has been recorded however, it is unspecified whether the genetic predisposition plays a role in ATLL development. In addition, ATLL may synchronize or follow other HTLV-1 induced non-neoplastic diseases being tropical spastic paraperesis. ATLL is grouped into different forms depending on the clinical presentations. The most common form is acute lymphoma and is characterised by leakaemic pictures, skin lesions and existence of systemic symptoms such as organomegaly and lymphadenopathy. In half of the patients, hypercalcemia is present and progression of another third may develop during the advancement of the disease. The chronic lymphoma is characterised by small volume lymphadenopathy and lymphocytosis that may be substantial for months or even years, hypercalcemia and organomegaly do not exist in this form. In smouldering, white blood cell count is normal and affected individuals are usually asymptomatic, however less than third of patients with the lymphoma do not show involvement of blood. In addition, ATLL patients are immunosupressed and that leads to the progression of opportunistic infections, which complicate the disease making more difficulties to be encountered during management. Strongyloides stercoralis epidemic in ATLL is persistent and may be severe and fatal. Patients with strongylodiasis and positive serology for HTLV-1 in their lymphocytes have clonal incorporation of the provirus and this parasite plays a major role in healthy carriers by allowing them to develop ATLL.
Prevalence of thrombocytopaenia and anaemia in ATLL is inconsistent. Neutrophilia and eosinophilia may be present. White blood cell count is increased with circulating atypical cells in acute and chronic form but they are not in smouldering because in this form the blood is not involved. The lymphocytes are of medium size and their chromatin is condensed with a convoluted nucleus classified as a flower cell, nucleoli is inconspicuous however if spotted is usually small. The cytoplasm is scant with a changeable degree of basophilia. A small number of blasts with prominent nucleolus and scattered chromatin may be present in the blood but often noted in other lymphoid tissues involved.
Bone marrow, lymph nodes and other tissues
The aspirate may present infiltration by lymphocytes in the bone marrow that have similar morphological characteristics to those in the blood. The trephine biopsy may or may not manifest lymphoid infiltration with degree involvement being inconsistent. Infiltration has the capability of being patchy or subtle in leukemic forms of ATLL. The infiltration pattern varies from occupying spaces to spread. Moreover, presence of osteoclasts with bone resorption is a definite feature in some hypercalcaemic cases.