Sandbox: ATL

or small volume lymphadenopathy, absence of hypercalcaemia and normal or only slightly raised lactate dehydrogenase (LDH) (less than twice the upper normal limit value). Patients with  unlike the chronic form, the white blood cell count is normal and there is no lymphocytosis and less than 3% atypical circulating lymphocytes. Less than a third of patients present with lymphoma with no evidence of blood involvement. There are several reports documenting disease progression to the acute form in patients with chronic and smouldering ATLL. Haemophagocytic syndrome as the first sign of transformation has been described in smouldering ATLL.12

In addition to the symptoms related to the neoplastic condition, patients with ATLL are immunocompromised and develop opportunistic infections that complicate the disease course and make its management more difficult. Infestation by Strongyloides stercoralis is frequent and may be severe and fatal. This association and the fact that a third of the patients with strongyloidiasis and positive serology for HTLV‐I have a clonal integration of the provirus in their lymphocytes, have led to the hypothesis that this parasite plays a role in the development of ATLL in healthy carriers.13

Most patients with medulloblastoma are initially asymptomatic. If left untreated, most of the patients with medulloblastoma will develop ataxia, nystagmus, and positional dizziness within a few months.

Acute ALT

The clinical course is aggressive with a median survival of less than 12 months in the acute and lymphoma forms. his is characterised by the presence of systemic symptoms, organomegaly, in particular lymphadenopathy, and a leukaemic picture. Skin lesions Hypercalcaemia with or without lytic bone lesions

Chronic

The chronic form is characterised by lymphocytosis which may be stable for months or even years, skin manifestations, no organomegaly

Smolud

smouldering ATLL are usually asymptomatic or manifest skin rashes that respond to topical steroids and/or lung infiltrates;

Complications

prognosis

high lactate dehydrogenase (LDH) high leukemic counts hypercalcemia poor clinical performance status