Adult T cell leukemia/lymphoma Flashcards
neoplasm of CD4+ /CD25+ Tcells, caused by human T c e l l
l y m p h o t r o p h i c v i r u s I ( H T LV- I ) .
ADULT T CELL LEUKEMIA / lymphoma
What CD cells are involved
CD 4/ CD25
Manifested by
skin infiltrates, hypercalcemia, visceral involvement, lytic bone lesions, and abnormal lymphocytes on peripheral smears
Mode of transmission
sex u a l i n t e r c o u r s e
p e r i n a t a l l y
e x p o s u r e t o b l o o d o r
b l o o d products (same as HIV).
Survival range
chronic forms, the median survival is ≥ 2 years.
In the acute and lymphomatous forms, it ranges from only 4 to 6 months.
Symptoms
fever, weight loss, abdominal pain, diarrhea, pleural effusion, ascites, cough, and sputum.
Skin lesions occurin 50% of patients with ATLL.
Single to multiple small,confluent erythem- atous,violaceous papules,±purpura;
form violaceous to brownishnodules;
papulosquamous lesions, large plaques, ± ulceration; trunk > face > extremities;
generalized eryth- roderma;poikiloderma;
diffuse alopecia.
Lymphadenopathy(75%)sparing mediastinal lymphnodes. Hepatomegaly (50%) and splenomegaly (25%)
Lymphadenopathy sparing mediastinal lymphnodes. Hepatomegaly and splenomegaly. which occurs more frequently w percentage of each
Lymphadenopathy(75%)sparing mediastinal lymphnodes. Hepatomegaly (50%) and splenomegaly (25%)
Most occurence in face/ trunk/ extremities.
In order of most to least.
Trunk> face > extremities
Labs
WBC normalto 500,000/μL.
Peripheral blood smears show polylobulated lymphocytic nuclei
(“ flower cells”).
Dermatopathology reveals lymphomatous infiltrates composed of many large abnormal lymphocytes,±giantcells,±Pautrier microabscesses. hypercalcemia in 25% at diagnosis of ATLL and in >50%during clinical course;
this is thought to be caused by osteoclastic bone resorption
Management
cytotoxic chemotx , response is <30%
combination of oral zidovudine and subcutaneous interferon-α
Allogeneic hematopoietic stem cell transplantation
