Diseases List

ID 158
Name NON-HODGKIN’S LYMPHOMAS
Cause
Signs Symptoms
Diagnosis
Investigations Investigations: 1. Lymph nodes biopsy- definitive diagnosis 2. Full blood count-pancytopania & findings of hypersplenism. 3. Autoimmune hemolytic anemia. 4. Bone marrow aspiration and trephine to define marrow involvement. 5. Cell membrane receptor studies 6. Staging investigation 7. HIVserology
Management Management:1-2 Management depends on the grade of the disease. Low-grade NHL: Low-grade lymphomas may be simply observed, only symptomatic treatment may be given. Indications for treatment include marked systemic symptoms, lymphadenopathy causing discomfort or disfigurement, bone marrow failure, spinal cord or other compression syndromes. Treatment options are: 1. Radiotherapy: This is useful in localised stage I disease, but is rare. 2. Chemotherapy: Chlorambucil oral therapy is well tolerated in most patients, but not curative. In younger patients i.v chemotherapy produces better quality of life but survival expectancy not increased. 3. Monoclonal antibody therapy: Humanised monoclonal antibodies, such as-anti-CD20 antibody rituximab can be used to target surface antigens on tumour cells, and induce tumour cell apoptosis directly. This induces a durable clinical response in up to 60% of patient when given alone. Rituximab also has been found acting synergistically when given in combination with chemotherapy comprising cyclophosphamide, vincristine & prednisolone (R-CVP) & is therefore recommended as first-line therapy. High-grade NHL: Intermediate or high-grade NHL should be treated immediately at initial presentation as thay are rapidly progressing and fatal. 1. Chemotherapy: Intravenous combination chemotherapy with the CHOP-regimen (cyclophosphamide, doxorubicin, vincristine and prednisolone) is used in majority (>90%) patients. 2. Radiotherapy: In high-grade NHL radiotherapy is also indicated for a residual localised site for bulk disease after chemotherapy. 3. Monoclonal antibody therapy: Rituximab (R) when combined with CHOP-regimen chemotherapy, increases the complete response rates and improve overall survival. R-CHOP is currently recommended as first-line therapy for those with stage II or greater diffuse larg-cell lymphoma. 4. Bone marrow transplantation (BMT): Autologus transplantation may be effective in patients with relapsed chemosensitive disease. Dosage: Please see in the therapeutic section or any text book.
Introduction These are a heterogeneous group of malignant disorders in which there is a monoclonal proliferation of lymphoid cells, the majority identifiable as B cell (85%) and a minority a cells. Non-Hodgkin’s lymphomas often merge with lymphoblastic and lymphocytic leukemias with which they have many features in common. The clinical presentation & course varying from indolent disease to rapidly progressive devastating illnesses
History
Etiology
Clinical Features Clinical features:1-23 A. Low-grade lymphomas usually present with painless lymphadenopathy, which may be isolated or wide-spread. The nodes are discrete & firm. There may be complains of some constitutional symptoms or patient may be symptom free. B. Intermediate & high grade lymphomas usually present with constitutional symptoms with adenopathy such as lassitude, weight loss, fever, sweating and anorexia. Patients with burkitt’s lymphoma frequently present with abdominal pain or abdominal fullness because of the predilection of the disease for the abdomen. Lymphomas may cause pressure effects as the area involved, such as dysphagia, breathlessness, vomiting, intestinal obstruction or ascites and limb oedema. Paraplegia or weakness of the legs may develop due to an extradural lymphoma compressing the cord. Bony involvement is indicated mainly by pain & occasional pathological fracture. Splenomegaly usually indicates its involvement. Paitents with HIV disease may develop non-Hodgkin’s lymphoma. Staging of NHL:* The Ann Arbor classification as outlined for Hodgkin’s disease is also applied for NHL. But incase of NHL it is frequently found widespread involvement (stage III or IV) at the time of diagnosis.
Preventions
Treatment
Complications
Prognosis
Types
Classification Classification: Precursor B: B cell lymphoblastic lymphoma Mature B: Diffuse large B cell lymphoma Mediastinal large B cell lymphoma Follicular lymphoma Small lymphocytic lymphoma Lymphoplasmacytic lymphoma Mantle cell lymphoma Burkitt lymphoma Marginal zone lymphoma - Malt type - Nodal - Splenic Mucosal tissue associated Precursor T: T cell lymphoblastic lymphoma Mature T: Anaplastic T cell lymphoma Peripheral T cell lymphoma Clinical grading:2 Low-grade: Small lymphocytic Small lymphocytic, plasmacytoid Follicular small cleaved cell Follicular mixed cell Intermediate-grade: Follicular large cell Diffuse small cleaved cell Diffuse mixed cell Diffuse large cell High-grade: Immunoblastic Small noncleaved (Burkitt’s) Small noncleaved (non-Burkitt’s) Lymphoblastic True histiocytic Other:. Mycosis fungoides (cutaneous T cell) Adult T cell leukemia/lymphoma Ty lymphocytosis Low grade tumours divide slowly, and may be present for many months before diagnosis and typically behave in an indolent fashion. High grade tumours divide rapidly, are typically present for a matter of weeks before diagnosis and may be life-threatening.
Observation
Pathology
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