Lymphoma/leukemia, lymphoblastic

A 60-year-old woman took dexamethasone 4 mg 8-hourly for dyspnea due to a precursor T lymphoblastic lymphoma-leukemia with bilateral pleural effusions and a large mass in the anterior mediastinum (130). She developed acute renal insufficiency and laboratory evidence of the metabolic effects of massive cytolysis. She received vigorous hydration, a diuretic, allopuri-nol, and hemodialysis. She recovered within 2 weeks and then underwent six courses of CHOP chemotherapy. The mediastinal mass regressed completely. She remained asymptomatic until she developed full-blown acute lymphoblastic leukemia, which was resistant to treatment. 

Anti-CD99 (M) Dako 1 20 MWER Recognition of virtually all primitive neuroectodermal tumors and Ewing s sarcomas labels roughly 50% of synovial sarcomas and malignant peripheral nerve sheath tumors also present in lymphoblastic lymphomas/ leukemias presenting in soft tissue... 

CD la is a transmembrane antigen normally found on cortical thymocytes and Langerhans cells. It is associated with beta 2 macroglobulin and may have a role in thymocyte development. It is found on a subset of precursor T lymphoblastic lymphoma/leukemia. 

Terminal deoxynucleotidyl transferase (TdT) is a DNA polymerase active during the process of immunoglobulin and T-cell receptor gene rearrangement early in a precursor B or T cell s life. Only normal early B and T lymphoblasts mark for TdT. The staining pattern is nuclear. TdT is a sensitive and specific antibody for lymphoblastic lymphoma/leukemia because only a small proportion of myeloid leukemia cases are positive (Fig. 6.13). 

CD20 and LCA may be negative in precursor B-cell lymphoblastic lymphoma/leukemia. 

Precursor B or T lymphoblastic lymphoma/leukemia has a similar overall cell size to Burkitt lymphoma but tends to have finer chromatin with inconspicuous nucleoli. These lymphomas have an immature phenotype and usually demonstrate positivity for TDT and/or CD34 with dim positivity for CD20 at best, and the absence of surface immunoglobulin allows a separation from Burkitt lymphoma. 

MTX is part of curative therapeutic schedules for acute lymphoblastic leukemias (ALL), Burkitt s lymphoma, and choriocarcinoma. It was also used in adjuvant therapy of breast cancer. High dose MTX with leucovorin rescue can induce about 30% remissions in patients with metastatic osteogenic sarcoma. MTX is one of the few antineoplastic drugs that can be safely administered intrathecally for the treatment of meningeal metastases and leukemic infiltrations (routine prophylaxis in ALL). In addition, MTX can be used as an immunosuppressive agent for the treatment of severe rheumatoid arthritis and psoriasis. 

Cytarabine is used in the chemotherapy of acute myelogenous leukemia, usually in combination with anthracyclines, thioguanine, or both. It is less useful in acute lymphoblastic leukemia and lymphomas and has marginal activity against other tumors. Myelosuppres-sion is a major toxicity, as is severe bone marrow hypoplasia nausea and mucositis may also occur. 

Precursor B-cell neoplasm Precursor B-lymphoblastic leukemia/lymphoma Mature (peripheral) B-cell neoplasms B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma... 

Cancers Multiple myeloma Non-Hodgkin s lymphoma Hodgkin s disease Acute myeloid leukemia Neuroblastoma Germ cell tumors Acute myeloid leukemia Acute lymphoblastic leukemia Chronic myeloid leukemia Myelodysplastic syndrome Myeloproliferative disorders Non-Hodgkin s lymphoma Hodgkin s disease Chronic lymphocytic leukemia Multiple myeloma... 

Acute lymphoblastic leukemia High-grade non-Hodgkin s lymphoma (i.e. Burkitt s) Intermediate risk... 

Vinblastine is used for severe lymphoblastic leukemia, Hodgkin s disease, non-Hodgkin s lymphoma, neuroblastoma, sarcoma, and other cancerous diseases. Synonyms of this drug are velban, eczal, and others. 

MM = multiple myeloma ATL = adult T-cell leukemia ALL = acute lymphoblastic leukemia CLL = chronic lymphocytic leukemia FCC = follicular center cell lymphoma DLC = diffuse large cell lymphoma TRB = T-cell rich B-cell lymphoma ALCL = anaplastic large cell lymphoma MALT = mucosa-associated lymphoid tissue-type lymphoma LC = lung cancer CMM = cutaneous malignant melanoma TCC = transitional cell carcinoma CSCC = cutaneous squamous cell carcinoma. 

Clinical applications include childhood acute lymphoblastic leukemia, choriocarcinoma, osteosar-com, non-Hodgkin s lymphoma and Burkitt s lymphoma. However methotrexate is also frequently used as an immunosuppressant in diseases such as psoriasis, rheumatoid arthritis and others. 

Corticosteroids suppress proliferation of lymphocytic cells, thus they are useful at combating acute lymphoblastic or undifferentiated leukemia of childhood, chronic lymphocytic leukemia, Hodgkin s lymphoma, other lymphomas. Therapy is often initiated with a steroid in combination with other agents. There is no evidence of cross resistance to unrelated agents. Mostly prednison is used however at appropriate dosages similar effects can be obtained with other glucocorticosteroids. 

Methotrexate is part of curative combination chemotherapy for acute lymphoblastic leukemias, Burkitt s lymphoma, and trophoblastic choriocarcinoma. It is also useful in adjuvant therapy of breast carcinoma in the palliation of metastatic breast, head, neck, cervical, and lung carcinomas and in mycosis fungoides. 

Etoposide is most useful against testicular and ovarian germ cell cancers, lymphomas, small cell lung cancers, and acute myelogenous and lymphoblastic leukemia. Toxicities include mild nausea, alopecia, allergic reaction, phlebitis at the injection site, and bone marrow toxicity. 

The major indication for L-asparaginase is in the treatment of acute lymphoblastic leukemia complete remission rates of 50 to 60% are possible. Lack of crossresistance and bone marrow toxicity make the enzyme particularly useful in combination chemotherapy, l-Asparaginase also can be used in the treatment of certain types of lymphoma. It has no role in the treatment of nonlymphocytic leukemias or other types of cancer. 

Lymphoma, breast and ovarian cancer, oat cell lung cancer Acute myelogenous and acute lymphoblastic leukemia, lymphomas Metastatic melanoma, sarcomas, Hodgkin s disease... 

Wilms tumor, Ewing s sarcoma, rhabdomyosarcoma, gestational choriocarcinoma, testicular tumors, lymphomas, melanomas Breast, ovarian, endometrial, bladder, thyroid cancers oat cell cancer of the lung Testicular, ovarian germ cell cancers, small-cell lung cancer, acute myelogenous and lymphoblastic leukemia... 

Acute lymphoblastic leukemia acute and chronic myelogenous leukemias Acute lymphoblastic leukemia Burkitt s lymphoma trophoblastic choriocarcinoma ... 

Ovarian adenocarcinoma, Breast carcinoma, Hodyhin s disease, non-Hodgkin s lymphoma, multiple myeloma, leukemia (acute lymphoblastic, acute myelogenous, acute monocytic, chronic granulocytic, chronic lymphocytic), mycosis fungoides, disseminated neuroblastoma, retinoblastoma PO 1-5 mg/kg/day. IV 40-50 mg/kg in divided doses over 2-5 days or 10-15 mg/kg every 7-10 days or 3-5 mg/kg twice a week. Biopsy-proven minimal-change nephrotic syndrome PO 2 5-3 mg/kg/day for 60-90 days. 

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