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Nekhodzhkinsky lymphoma

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Nekhodzhkinsky lymphoma
Clinic, diagnosis, treatment

Nekhodzhkinsky lymphoma integrate group of the diseases which are characterized by primary local, mainly extra marrowy tumoral growth of lymphatic fabric. These lymphoid tumors differ on a morphological picture, features of a clinical current, the forecast.

Epidemiology
The standardized indicators of incidence lympho-and reticulosarcomas fluctuate within 2 — 6,9 at men, 0,9 — 5 at women.
Get sick with Nekhodzhkinsky lymphoma of the man considerably more often than women, their age by the time of establishment of the diagnosis fluctuates in big limits.

Etiology and pathogeny (see Hemoblastoses)

Classification
Options of nekhodzhkinsky lymphoma (lymphosarcomas) establish according to the morphological WHO classification which are correlated to degree of the zlokachestvennost provided in "The international working formulation of nekhodzhkinsky lymphoma for a clinical use".

Nekhodzhkinsky lymphoma of low degree of a zlokachestvennost:
— lymphocytic, diffusion type;
— pro-lymphocytic, nodulyarny type;
— limfoplazmotsitarny.

Nekhodzhkinsky lymphoma of intermediate degree of a zlokachestvennost:
— prolimfotsitarno-lymphoblastoid, nodulyarny type;
— pro-lymphocytic, diffusion type;
— prolimfotsitarno-lymphoblastoid, diffusion type.

Nekhodzhkinsky lymphoma of high degree of a zlokachestvennost:
— immunoblast, diffusion type;
— lymphoblastoid (macro - micro, with the twisted and not twisted kernel), diffusion type;
— Berkitt's tumor.

In separate sections of the WHO classification fungoid mycosis, reticulosarcomas (on modern concepts the most part of the last is provided by lymphoid tumors and insignificant — histiocytic option), a plasmacytoma, not classified lymphoma are allocated.

Formation of pseudo-follicular structures which unlike true follicles are defined as in cortical, and brain layers of a lymph node is characteristic of a nodulyarny lymphosarcoma, have the big sizes, indistinct contours and rather monomorphic cellular structure.
Diffusion type of growth svoystven to all options of nekhodzhkinsky lymphoma. The diffusion lymphocytic lymphosarcoma is characterized by total growth of cells like small lymphocytes, infiltriruyushchy walls of blood vessels that leads to full deleting of normal drawing of a lymph node. Similar changes are similar revealed at HLL in this connection crucial importance in differential diagnosis has a complex of kliniko-hematologic signs (temporary parameters, localization of tumoral process, a picture of peripheral blood, marrow, etc.).

The combined proliferation lymphoid is inherent to a diffusion limfoplazmotsitarny lymphosarcoma and plasmocytes, also plazmatizirovanny lymphocytes meet. Changes at this option of a lymphosarcoma are similar to the picture which is found at Valdenstrem's macroglobulinemia; the disease is often combined from various type by monoclonal gammapathies.

The diffusion pro-lymphocytic lymphosarcoma is characterized by growth of cells of the big sizes, than the small lymphocytes having kernels of rounded or irregular shape ("dissect" kernels) in which 1 — 2 nukleola are looked through. Chromatin of a kernel is less dense, than at a small lymphocyte. At process generalization peripheral lymph nodes, a liver, a spleen, marrow most often are surprised (in 25 — 45% of cases). The five-year survival makes 63 — 70%. Modern treatment provides practical recovery of the most part of patients in the I stage of process.
At a diffusion lymphoblastoid lymphosarcoma growths of cells like lymphoblasts among which occur also microgeneration are found. Cells with kernels mozgovidny (twisted, konvalyutny) forms can be found. They are more often observed at children
at localization of process in lymph nodes of a mediastinum also have, as a rule" the T-cellular nature. Existence of a large number of cells in a condition of a mitosis, the breaking-up cells is characteristic of a lymphoblastoid lymphosarcoma.

The diffusion immunoblast lymphosarcoma differs in massive growth large one - or multinucleate cells with big is central the located nukleoly and plentiful zone of basphilic cytoplasm. The large number of mitoses, the perishing cells comes to light. Along with immunoblasts the considerable number of plasmocytes is found. The forecast is adverse, the five-year survival of patients fluctuates from 21 to 32%.

Berkitt's lymphoma differs in monomorphic proliferation of blast cells of lymphoid type with hyper basphilic often vakuolieirovanny narrow cytoplasm. On this background typical, though not specific, feature is presence of large macrophages who create a picture of "the zayezdny sky". There is an opinion on proximity of cells of a lymphoma of Berkitt to partially blasttransformirovanny V-lymphocytes. Unlike other forms of nekhodzhkinsky lymphoma the tumor is initially localized mainly
ekstranodalno.

At a reticulosarcoma (a histiocytic lymphoma), rather seldom found tumor, growths of cells with morphological and functional features of macrophages, the large cells of the roundish or extended form containing the light kernel of the average sizes of a fabiform form about 1 — 2 nukleolam surrounded with quite wide rim of poorly basphilic cytoplasm are found. The part of cells finds ability to phagocytosis. Cells are characterized by high activity of nonspecific esterase, ability to allocate a lysozyme, lack of specific markers.

The nondifferentiable type is characterized by a .proliferation of sharply anaplazirovanny cells having large irregular shape the kernel surrounded with rather narrow zone of slabobazofilny cytoplasm. Believe that the part of these tumors has a lymphoid origin.

Along with the given classification use also others. So, some authors suggest to subdivide lymphosarcomas depending on primary localization of process; the term "lymphocytoma" emphasizes high quality of a course of the extra marrowy tumors consisting mainly of mature forms of the small lymphocytes (or lymphocytes and prolymphocytes) forming structure of nodulyarny growth. Therefore they are allocated from nekhodzhkinsky lymphoma of low degree of a zlokachestvennost in separate group of lymphoid tumors.

Progressing of nekhodzhkinsky lymphoma can be followed by change of morphological option of a disease, transformation of a nodulyarny lymphosarcoma in diffusion.

Approximate formulation of the diagnosis:
1. A lymphocytic lymphosarcoma with defeat of a cervical lymph node (on the one hand); diffusion type (IA stage).
2. A pro-lymphocytic and lymphoblastoid lymphosarcoma, with defeat of cervical and axillary lymph nodes, spleens, fever, weight loss; modular type (IIIB stage).
3. A lymphoblastoid lymphosarcoma with defeat of peripheral, mediastinal and retroperitoneal lymph nodes, marrow, spleen, liver and lungs, exhaustion, periodic fever, diffusion type (IVB a stage).
4. A lymphocytoma with defeat of a lymph node of a neck (on the one hand); modular type (IA stage).


 
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