OVERVIEW
OVERVIEW
Adult T-cell leukemia/lymphoma (ATL) is a malignant monoclonal proliferative disease of T cells caused by human T-cell leukemia virus type I. It is a rare type of lymphocytic leukemia/lymphoma with T-cell involvement. It is a rare and specific type of lymphoblastic leukemia/lymphoma with T-cell involvement. Clinically, it can be divided into acute, chronic, occult and lymphoma types. Gao Yueqing classified the disease into 5 types, and categorized the chronic type, occult to acute type as acute transformation type. It is characterized by generalized lymph node enlargement, hepatomegaly, splenomegaly, skin bone marrow damage, petaloid nucleated lymphocytes in peripheral blood, osteolytic damage and hypercalcemia.
Whether medical insurance
Yes
Department
Hematology
Clinical Symptoms
Clinical manifestations are varied, and common symptoms include general malaise, fever, cough, abdominal distension, malaise, and diarrhea. However, almost all patients have hepatomegaly, splenomegaly and enlarged lymph nodes, often with skin damage.
Hazards
The disease has a very poor prognosis and a low long-term survival rate.
Examination
Routine blood, bone marrow examination, immunophenotyping, serologic examination, molecular biology examination, tissue biopsy, CT examination, endoscopy and so on.
Diagnosis
Diagnosis is based on clinical manifestations such as enlarged lymph nodes, combined with blood routine, serologic examination, tissue biopsy, and immunophenotype.
Treatment principle
At present, there is no specific treatment measure. Chronic and cryptogenic types can be left untreated if there are no obvious clinical manifestations and the blood picture is generally normal. Acute type and lymphoma type can be treated with radiotherapy, chemotherapy and hematopoietic stem cell transplantation.
Curability
The disease has a poor prognosis and low long-term survival rate, while a few patients can obtain long-term survival after active treatment.
Dietary advice
Give a high-protein, high-vitamin, easy-to-digest diet.
Causes
Causes
The disease is caused by human T-cell leukemia virus type-1 infection.
Symptoms and Diagnosis
Typical symptoms
1. Acute type has a rapid onset and is characterized by leukemic manifestations, often with systemic symptoms, mostly with enlarged lymph nodes, hepatomegaly and splenomegaly, often accompanied by skin lesions and hypercalcemia, skin lesions include erythema, plaques or nodules, larger nodules may have ulcers, and hypercalcemia manifests itself as malaise, loss of appetite, vomiting, irritable thirst, polyuria, and fuzzy consciousness. 2. Lymphomatous type with histologically confirmed lymph node infiltration, no leukemia manifestations. Patients with advanced disease present similarly to the acute phase, hypercalcemia is less common, and skin lesions are common.3. Chronic type has lymphocytes >4×109/L, T lymphocytes >3.5×109/L, LDH 2 times higher than the upper limit of normal, no hypercalcemia, no CNS, bone, gastrointestinal tract infiltration, and no pleural or peritoneal effusion.4. Smoldering type has lymphocytes <4×109/L, pleomorphic lymphocytes >5%, patient may have skin and lung damage, no hepatomegaly, splenomegaly, lymph node enlargement, no hypercalcemia. There is no cerebrospinal fluid, bone, gastrointestinal tract infiltration, pleural effusion or abdominal effusion.
Diagnostic basis
1. Clinical manifestations: onset in adults, superficial lymph node enlargement, no mediastinal or thymic tumors. 2. Laboratory tests: peripheral blood leukocyte count is often increased, polymorphonuclear lymphocytes account for more than 10% of the total, which are of the T-cell type, with surface markers of mature T-cells, and the serum is positive for HTLV-1 antibody. 3.
Treatment
Treatment guidelines
Chronic and smoldering types can be left untreated if there are no obvious clinical manifestations and the blood picture is generally normal. The acute and lymphoma types are treated with radiotherapy, chemotherapy and hematopoietic stem cell transplantation.
Drug treatment
1. Mostly chemotherapeutic drugs. 2. Tac antibody. 3. Antifungal drugs can be given to prevent fungal infections in chemotherapy for acute type, lymphoma type and chronic type of ATL with poor prognosis.
Radiotherapy
Chemotherapy may be given for newly diagnosed acute type, lymphoma type and chronic type of ATL with poor prognosis. Prophylactic intrathecal chemotherapy may also be advocated for smoldering types of ATL.
Other treatments
Hematopoietic stem cell transplantation.
Prognosis
The disease has a poor prognosis and low long-term survival, with prognostic correlates: poor general condition; hyperlactate dehydrogenasemia; age >40 years; multiple sites of involvement; hypercalcemia; immunophenotype; Ki-67 >18%.
Nursing care
Daily care
1. Maintain appropriate greenhouse degree and ventilate frequently.2. Patients should wear breathable and cotton clothes, and should be kept warm if there are chills.3. Keep patients’ organism clean to prevent the spread of bacteria in the body, and do a good job in oral care, perineal-anal care to prevent all kinds of infections.4. Patients should follow the doctor’s instructions for medication, adhere to regular medication, and have regular outpatient rechecking.5.
Dietary management
Give high protein, high vitamin, easy to digest diet, avoid spicy stimulating food.