HOMO SAPIENS DISEASES - LYMPHOPROLIFERATIVE DISORDERS (LPD)

Table of contents :


  • B-cell leukemias/lymphomas
  • Hodgkin's disease (HD) or lymphoma / malignant lymphogranuloma / granulomatous lymphoma
  • non-Hodgkin's lymphomas / leukemias (NHL)
  • B cells acute lymphoblastic or lymphocytic leukemia (B-ALL)
  • mature peripheral B-cell neoplasms
  • plasma cell dyscrasias (PCD)
  • Oligoclonal lymphomas
  • T-cell leukemias/lymphomas
  • T-cell acute lymphoblastic leukemia (T-ALL)
  • T-cell large granular lymphocyte leukemia (T-LGL)
  • aggressive NK-cell leukemia
  • adult T-cell lymphoma / leukemia (ATL / ATLL) (HTLV-1+)
  • peripheral T-cell lymphoma (PTCL), unspecified
  • angioimmunoblastic T-cell lymphoma (angioimmunoblastic lymphadenopathy (AILD)-like)
  • adult T-cell lymphoma / leukemia (ATL / ATLL) (HTLV-1+)
  • anaplastic large cell lymphoma (T- and null cell) (ALCL)
  • mycosis fungoides/Sézary's syndrome
  • primary cutaneous ALCL (pcALCL)
  • extranodal nasal/nasal type NK/Tcell lymphoma
  • blastic NK-cell lymphoma
  • myeloid/NK cell precursor acute leukemia
  • enteropathy-associated T-cell-lymphoma (EATCL)
  • subcutaneous panniculitis-like T-cell lymphoma (SCPTCL)
  • hepatosplenic T-cell lymphoma (HTCL)
  • mixed lineage leukemias
  • lymphomas in the immunosuppressed patient
  • AIDS-related lymphomas (ARL)
  • post-transplant lymphoproliferative disorder (PTLD)
  • Web resources


  • lymphoproliferative disorders (LPD) / lymphomas (i.e.cancers beginning in a lymphoid tissue) Genetic lesion involves a committed lymphocyte precursor. If in such a leukosis neoplastic lymphocytes are found into blood, neoplasm is named  lymphatic, lymphoblastic, lymphocytic, lymphogenous, or lymphoid leukemia, otherwise it is said to be an aleukemic or subleukemic leukosis or "leukemia" / leukopenic or aleukocythemic leukemia (total white blood cell count in the peripheral blood is either normal or below normal). It is classified according to degree of cell differentiation as acute or chronic (terms no longer referring to duration of disease). Leukemias cause hyperviscosity syndrome. Neoplastic lymph nodes are usually wooden and painless : diagnosis is made by lymph node biopsy. Primary lymphoma have not to be confused with metastatic localizations. Average life expectancy after diagnosis varies from some weeks (highly aggressive) to some years (indolent). Pathogenesis : most lymphoid malignancies are initiated by specific chromosomal translocations between immunoglobulin (Ig)/T cell receptor (TCR) gene segments and cellular proto-oncogenes. In many cases, illegitimate V(D)J recombination has been proposed to be involved in the translocation process. 2 distinct mechanisms are involvedref : Tumorigenic cell morphology : Tumorigenic cell types :
    Symptoms & signs : pentad of telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting.
    Therapy : bortezomibref1, ref2, ref3.

    Localizations : Epidemiology : expected rate of 4.2 cases of leukaemia for every 100,000 kids over a 5-year period
    Aetiology : Laboratory examinations : Therapy : Prognosis : ILSG classification The International Prognostic Index (IPI) is based on ...., and renders it possible to identify 4 risk groups corresponding to the number of adverse parameters : In young patients an age-adjusted IPI based on tumour stage, serum LDH level, and performance status also identified 4 risk groups. In both models, the increased risk of death was the result of both a lower rate of complete responses and a higher rate of relapses. The IPI may also be effectively applied to patients presenting with lymphoma subtypes other than DLCL [3, 6]. The IPI and the age-adjusted IPI have proven significantly more accurate than the Ann Arbor classification in predicting long-term survival. Since the serum b2-microglobulin level was measured inonly a few centres for patients included in the IPI project, the IPI could not incorporate this important prognostic indicator. However, the MD Anderson Cancer Center team has developed models that included b2-microglobulin level and other important parameters, and proposed an index based on LDH and b2-microglobulin level that has proven helpful indistinguishing between good-risk and poor-risk patients, regardless of their lymphoma subtyperef. The IPI incorporates in its definition only surrogate markers of profound alterations of the cellular biology of tumour cells, particularly the mechanisms involved in the control of mitosis or of the host response machinery to the cancer. Since the time of its description, and in the future, biological or genetic alterations have been, and will be, described that may replace all or some of the classical parametersref. These putative parameters include gene alterationsof cell-cycle regulator proteins (Bcl-2, p53, Rb, p16, p21), cytokines (TNF, IL-6), adhesion molecules (CD44, ICAM-1), angiogenic peptides (VEGF), and transmission factor expression.

    Complications : late non-neoplastic events were found in 46% of the 757 NHL patients. At 15 years, the cumulative incidences of cardiac disease and infertility were 20% and 29%, respectively. Renal insufficiency (11%), acquired hypertension (8%), and disabling neuropathy (13%) were also frequent. Salvage treatment was a risk factor in most cases. Smoking, age > 50 years during treatment, and preexistent hypertension were the main risk factors for cardiovascular disease. In-field radiation therapy (RT) was related to hypothyroidism, lung fibrosis, hypertension, gastrointestinal toxicity, and renal insufficiency but not to cardiovascular events. Autologous stem cell transplantation and cisplatin- and MOPP-containing therapies were associated with infertility and renal insufficiencyref
    Standardized response criteria : in 1987, Dixon et alref emphasized the need for uniform reporting of end points in clinical trials of patients with non-Hodgkin's lymphomas (NHL); of particular importance were the complete remission rate, survival, time to treatment failure, and time to relapse of complete responders. Their recommendations were met with controversy that remained unresolvedref. Several USA lymphoma investigators from National Cancer Institute (NCI)-sponsored cooperative groups, the NCI, and the pharmaceutical industry collaborated in an effort to resolve the issues regarding response assessment in NHL. The result was a preliminary document that was subsequently reviewed and approved by European lymphoma experts (Horning S, Cheson B, Peterson B, et al: Response criteria (RC) and quality assurance (QA) of responses in the evaluation of new therapies for patients with low-grade lymphoma (LGNHL). Proc Am Soc Clin Oncol 16:18a, 1997 (abstr 61); Grillo-López AJ, Horning S, Cheson B, et al: Development of response criteria (RC) for low-grade or follicular lymphomas (LG/FNHL) and application in a 166 patient study. Exper Hematol 25:732, 1997 (abstr 17)). Eventually, a workshop was held at the NCI on February 25 to 26, 1998, with a subsequent meeting on May 16, 1998, to come to consensus on a standardized set of guidelines for response assessment in adult patients with indolent and aggressive NHL. Response criteria for NHL used by International Cooperative Groups : In conclusion, the most important end points of clinical trials in NHL should be overall survival and failure-free survival (time to treatment failure and event-free survival). In phase II trials, particularly in the setting of relapsed and refractory disease where the activity of a new agent may be the most important objective, response rates are important and may provide support for approval by regulatory agencies. On the other hand, the goals of most phase III trials are to identify therapies that will prolong the progression-free survival, if not the overall survival, of the treated patients. Response rates will continue to be ambiguous and subject to considerable controversy as long as we are required to base guidelines on consensus opinion of clinical data rather than on more precise and reliable measures of minimal residual disease. Moreover, particularly in the follicular NHL, incremental increases in response rates have not uniformly translated into prolonged time to treatment failure or survival. A residual mass in a patient with a large-cell lymphoma, in whom the disease may have been cured, has different implications from a patient with a follicular lymphoma, in whom the mass may remain stable for months but will inevitably progress. To improve response assessment, we strongly encourage the use of SPECT gallium scanning in patients with large cell-lymphoma
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