Hematosarcomas

There is a bunch of aleukemic leukoses at which tumour cells in an osteal brain of appreciable representation have no.

Classification of aleukemic hemoblastoses:

1. Lymphocytomas
2. Lymphosarcomas
3. Myeloblast sarcomas
4. Erythroblast sarcomas
5. Monoblast sarcomas
6. Plasmablast sarcomas
7. Macrophagal sarcomas:
   • actually macrophagal sarcoma
   • a fibrosing cancerous histiocytoma
8. Not differentiated hematosarcomas

Hematosarcoma growth can be nodular (in the form of a lobe). In this case kernels disjoined. Cells morphologicallies correspond to the normal cells which are settling down in the centre of a follicle of a lymphonodus. As a rule, it is the V-cellular nature lymphocytes. Diffusive growth shows in the form of a blurring of a drawing of a lymphonodus. Cells same, different under the form and the dimensions of cores.

Criteria of the tumoral nature:

1. The cytologic, histological picture
2. Results immunemorphological researches (presence of fields of the cells containing on a surface monoclonal markers)

Lymphosarcomas

Lymphosarcomas - extra bone marrow tumours from blast cells lymphatic nature - lymphoblasts (or lymphoblasts and prolymphocytes).

Clinic very much polymorphic. In one cases the augmentation of a lymph node (lien), a thyroid gland lobe, etc., having a dense consistence, mobile, painless happens the first symptom of disease. In other cases the intoxication picture, development of an autoimmune hemolitic anaemia, a polyarthritis, an eczema similar enanthesises can precede tumour detection. Disease can begin with a syndrome of a prelum of veins and absorbent vessels with disturbance of function of an organ.

The picture of blood has no specific features: red blood (except for a hemolysis and diffusion of process to KM) does not suffer, the quantity of leucocytes can be normal or slightly raised without essential changes in the formula. T - lymphoblasts sarcomas can be accompanied by an eosinophilia in bloods and KM. The ESR can be both normal, and raised. In case of a leukemisation of a lymphosarcoma the blood picture is similar to that at the acute leukosis characterised by depressing of normal sprouts of a hemopoiesis, a blastosis of blood and KM. On occasion lymphosarcomas in blood the absolute lymphocytosis, and in KM - high percent of end-stage lymphocytes is observed.

Diagnostics. The thought on a hematosarcoma arises when there is somewhere a dense tumour. As the indication to a biopsy such tumour serves in a lymphonodus, on a skin, in a stomach, an intestine, tonsils, etc. Absence of clinical signs speaks more likely in favour of tumoral, instead of the inflammatory nature of disease. In all cases of a biopsy it is necessary to do impresses and smears of the received stuff since in the cytologic preparation it is easy to establish the blast nature of a tumour.

The lymphosarcoma passes following stages. 1. Restriction of growth of a tumour only a tissue of a lymphonodus or a tissue of other primary localisation (a bone, a fatty tissue, a skin, a stomach, etc.). 2. Metastasises out of a tissue of primary localisation. 3. Tumour germination in surrounding tissues, coalescence to surrounding fat. 4. Lymph drainage disturbance - edema appearance distally located fields. 5. Medullar drift (leukemisation). For progressing lymphosarcoma metamorphosis of the nodular form into the diffusive is characteristic.

In the separate form the Burkitt's lymphoma is secured. Meets mainly at the population of tropical Africa, amazes children of 2-15 years, case rate peak - 4-6 years. Clinical feature - frequency of a lesion of facial bones of a skull, in 1st turn of the top jaw and an orbit. At tumour diffusion organs of an abdominal lumen are often amazed. Metastasises in the excitatory system, especially in a spinal cord are frequent. Cells of a tumour of a blast type, it is frequent with a cytoplasma and even kernel vacuolation; against their diffusive proliferation not tumoral macrophages giving to a tissue specimen a picture of "the star sky” settle down. Quite often at disease Epstein-Barr virus in a genome of tumour cells a the assumption of a virus parentage of a tumour is found. In last to year descriptions of not African Burkitt lymphoma have become frequent.

At a lymphosarcoma of peripheric lymphonoduses more often a primary tumour - on a neck in supraclavicular space. Metastasises - in the surrounding lymphonoduses, symmetric range of a neck, a mediastinum.

The lymphosarcoma of a thymus at children has the expressed malignance. Process quickly metastasizes in covers of a brain and in testicles.

The stomach lymphosarcoma causes gravity in a gaste, pains after meal, sometimes a bleeding. Roentgenologically the tumour is indistinguishable from a cancer - the biopsy is necessary at generalised discoordinatory syndromes. Treatment operative

Intestine lymphosarcoma - more often at men of 30-40 years. Histologically - a monomorphic of cells - lymphoblasts. The tumour in an intestine side is localised, involving regional lymphonoduses. There is a clinic of intestinal impassability, an abdominal pain. Metastasises for limens of an abdominal lumen are rare. The good effect gives operative treatment.

The lien lymphosarcoma is accompanied by organ augmentation. Gravity in left hypochondrium, palpatory - dense edge of a lien. The lien punctate taps prevalence of blasts. Sometimes the lien lymphosarcoma causes the hypersplenism phenomenon. In blood can be without changes, sometimes - an absolute lymphocytosis, basket cells. The KM is normal. Treatment - a splenectomy.

The skin lymphosarcoma - meets was very rarely separately. Morphologicallies it is diffusive growth of layers of young lymphoid cells in a false skin. The skin acyanotic and cold, has ligneous density on the circumscribed field of a skin. Treatment - radial therapy.

The lung lymphosarcoma is diagnosed as a rule late. At a widespread lesion causes an original pneumonia: the dry tussis, an accruing dyspnea, a high fever, “an acyanotic cyanosis” (acyanotically-grey colour) skins, poor auscultative data. Antibiotics do not help (only for the short season reduce temperature). Respiratory insufficiency progresses. Unlike respiratory insufficiency of inflammatory character, at a lymphosarcoma patients, hardly suffering from a dyspnea, do not accept the forced sitting position, and lay. Roentgenologically - reminds a miliary tuberculosis. The diagnosis is put on a lung biopsy. Diffusive growth of the young lymphoid cells forming large proliferates, infiltrative an intersticial tissue of a lung, alveolar septums is taped.

The myocardium lymphosarcoma causes a subitaneous and quickly progressing left ventricular failure, stagnation in lungs, a liver, edemas. The first symptoms - a tachycardia, a dyspnea at walking For some weeks> insufficiency of 2 degrees. The diagnosis is established on a lymphocytosis in peripheric blood.

The lymphosarcoma of a root of tongue clinically shows quickly growing tumour of a root of tongue, for some days leading to a dislocation of tongue which ceases to be located in an oral cavity. The diagnosis - by results of a biopsy.

The lymphosarcoma of tonsils - slowly progresses augmentation of tonsils. The biopsy taps a lymphosarcoma.

The lymphosarcoma of nephroses as the isolated process meets was rarely. Metastasises since define a disease picture - back pains and an anuria are worthy. The palpation finds dense nonmotile tumours. Treatment - radial therapy.

Testicle lymphosarcoma - a painless dense tumour at a palpation.

Along with clinically outlined lymphosarcomas, which first symptom there is a tumour detection, there is a bunch atypically educing lymphosarcomas - are often taped in a generalised stage, at local growth it is difficult to define their tumoral or reactive nature. There are following variants:

Gipertermicheski-toxic type

Hemolitic type

The toxicosis without the obvious tumoral locus or the expressed hemolitic syndrome is expressed. A hemolysis thus the immune. To diagnose a lymphosarcoma as an intoxication and hemolysis source it is possible only on the basis of a biopsy of the enlarged lymphonodus found at repeated X-ray inspections since often enlarged lymphonoduses settle down in a thorax or an abdominal lumen.

Hydropic variant

It is accompanied by causeless edemas of extremities against moderate augmentation of the conforming regional lymphonoduses. Function of nephroses, hearts remains normal. There is no stagnation in lungs. Use of diuretics does not give effect. In a biopsy sample of lymphonoduses - prevalence of lymphoblasts and prolymphocytes.

Pseudoimmunoblast variant

Represents especially big difficulties for diagnostics. Reminds both an infectious mononucleosis, and a lymphosarcoma. Augmentation of lymphonoduses of many bunches, they dense, quite often painless, are surrounded by hydropic tissues, the intoxication picture is expressed - the temperature, a sweating, a weight loss is raised. In impress Biopsed of a lymphonodus immunoblasts, prolymphocytes, plasmocytes prevail. Almost full disturbance of structure of a lymphonodus.

Myeloblast sarcoma

As the independent form meets was rarely. As a rule, for a short while advances development acute myeloblast a leukosis, or, more often, educes in an end-stage of chronic myeloses or in the course of a tumoral progression of acute leukoses. Has the name "chloroma" in connection with a virescent coloration on a cut, the caused myeloperoxidase. But it is no by less differentiated myeloid cells, than myeloblasts and promyelocytes. Establish an accessory of last type of sarcomas to a granulocytic number by means of histochemical reaction to a chloroacetate esterase, and on occasion only a submicroscopy. Chloromas are localised by of an island in the field of orbits, flat bones of a skull and other ranges bound to an osteal tissue. Quite often - in ovaries, nephroses, etc. Treatment - by principles acute myeloblast a leukosis.

Erythroblast sarcoma

Meets rarely. It can be localised in lymphonoduses. Cellular structure - eritro karyocytes all stages of maturing and undifferentiated blast cells, myelocytes and promyelocytes. Badly gives in to chemotherapy.

Monoblast sarcoma

It is accompanied by the expressed intoxication and quite often hyperthermia. The diagnosis is established on positive reaction of tumour cells to a naphthyl esterase. Without treatment becomes generalised

Macrophagal sarcoma

Activity of cells about which speaks presence at their cytoplasma of bloody elements, cellular detritis, alien particles is presented by macrophagal blasts with phagocytal. In connection with haemophagocytal activity of cells the macrophagal sarcoma, at all not metastasizing in KM, can become complicated a cytopenia. The macrophagal sarcoma can grow everywhere, with an early dissimination on organs. Causes a serious intoxication, a hyperthermia.

The fibrosing cancerous histiocytoma - arises on extremities, after a trauma, infiltrate soft tissues is more often. Cells of the macrophagal nature amounting it differ in the big dimensions, an abundant activly phagocytizing cytoplasma, a young structural kernel. It is a lot of fibroblasts.

Lymphogranulomatosis

This primary tumoral disease of lymphatic system. Process arises unicentric and extends metastatic by. In 1832г. Hodgkin has described 7 patients with a lesion of lymphonoduses and liens. Men compound 60-70 % of the diseased. It is diagnosed for humans of all age, including newborns. Rising of a case rate at humans is peculiar is more senior 50 years and in an age-grade of 16-30 years. In 3 times meets in monogynopaediums where have already been registered sick of a lymphogranulomatosis is more often.

The aetiology is unknown. The role of the etiological factor was consistently shunted micobacteria of a tuberculosis and many other things to originators, including viruses. Already in 30th years Davydovsky carried disease to diseases of the tumoral nature.

Tumour cells are: huge Sternberg cell and Hodgkin's cell. The aneuploidy and clonality Sternberg cell - tumoral qualities are proved. Concerning a parentage of this cell there is an assumption about monocytic - a macrophagal parentage.

Lymphogranulomatosis originality as tumoral disease - absence of mutual transition in other diseases of this bunch. Development of leukoses at sick of a lymphogranulomatosis is surveyed as apposition of the second disease.

Lymphogranulomatosis substrate - the polymorphocellular granuloma formed by lymphocytes, reticular macrophages, neutrophils, eosinocytes, plasmocytes and a fibrous tissue. Lymphogranulomatosis the tissue forms in the beginning separate fine nodules in a lymphonodus, and then, progressing, supersedes a normal tissue of a lymphonodus and erases its drawing. Histological feature of a granuloma are huge Sternberg cell. These are large cells 25 microns in diameter and more, keeping 2 and more oval or roundish kernels. The chromatin of kernels tender, is located in regular intervals. A nucleolus large, accurate. Hodgkin's cell - large uninuclear cells.

Histological classification:

• Lymphohistiocytic variant. The proliferation mature leukineferonsи hystiocytes is characteristic. Eosinocytes, neutrophils, plasmocytes are not numerous. The expressed locuses of a sclerosis and a necrosis it is not observed. Sternberg cell it is not enough.
• Nodular sclerosis. The correct bands of collagen dividing a tumour on fields of the roundish form are formed. The tumoral tissue in the nodule centre can consist from mature leukineferons among which settle down especially large Sternberg cell with a wide foamy cytoplasma and set of fine kernels.
• Mixedcellular variant. Cells very much polymorphic. Sternberg cell, eosinocytes, neutrophils, leukineferons, hystiocytes, fibroblasts, bands of a diffusive fibrosis. Cellular clumps and sclerosis fields are usually located irregularly, that gives to histic growths a motley kind.
• Variant with lymphoid attrition. Unites to 2 morphologicallies various forms on the basis of full absence leukineferons. The diffusive sclerosis is accompanied by sharp prevalence of growths of rasping bands of a fibrillar copulative tissue with abaissement of amorphous albuminous masses among which on separate fields insignificant clumps of cells settle down, mainly Hodgkin's cell, Sternberg cell, fibroblasts. Predominances of reticular macrophages, atypical both typical Sternberg cell and moderately expressed fibrosis are inherent to a reticular variant.

Change of histological variants at disease development:

The form with prevalence of an adenoid tissue> the admixed cellular variant> the form with adenoid tissue depressing.

Clinic. It is rather various. Beginning in lymphonoduses of this or that bunch, pathological process can extend on all organs, be accompanied by variously expressed symptoms of an intoxication. The primary lesion of this or that organ or system defines a clinical picture of disease. The augmentation of lymphonoduses becomes the first implication of a lymphogranulomatosis usually. In 60-75 % of cases process begins in is cervical-supraclavicular ganglions. The enlarged lymphonoduses are mobile, densely elastic, are not soldered to a skin. Gradually, being sometimes enlarged, they merge in large conglomerates. 5-25 % of patients have pains in lymphonoduses after alcohol reception. At 15-25 % of patients process begins with augmentation of lymphonoduses of a mediastinum. This augmentation can be casually found at photoroentgenography or to show in serotinal terms when the conglomerate dimensions are appreciable, tussis, a dyspnea, is rarer - pains behind a breast bone. The lesion of lymphonoduses of a mediastinum is typical for a sclerosis. Disease is diagnosed late, and the forecast bad. At 5-10 % of patients process begins acutely with a fever, night sweats, a fast weight loss. Usually in these cases the insignificant augmentation of lymphonoduses appears later; disease is accompanied by an early leukopenia and an anaemia. Often such beginning associates with a phase of lymphocytic attrition - the bad forecast.

In the developed implications probably lesion of all lymphoid organs and all organs and systems. The lien is amazed in 25-30 %. The phenomena of a hypersplenism are not present. The Most frequent among extranodal localisations - a pulmonary tissue. Infiltrative growth from mediastinum lymphonoduses, development of the separate locuses or diffusive infiltrates, sometimes with disintegration and formation of lumens is possible. The lesion of a pulmonary tissue usually is not accompanied by physical symptoms. Sternberg cell in a sputum are found was rarely. Often there is a fluid clump in a pleural cavity. The tumour in mediastinum lymphonoduses can sprout in a pericardium, an esophagus, a trachea. Osteal system. Vertebra, then a breast bone, pelvic bones, ribs are more often amazed. Involving in process of bones shows pains. The lesion baking because of its big compensatory possibilities is found late. The liver usually is enlarged, raises activity of an alkaline phosphatase, Serum albumin decreases. GASTROINTESTINAL TRACT, as a rule, suffers again in connection with squeezing or germination of a tumour from the struck lymphonoduses. A CNS. Lesions are localised in meninxes, give serious neurologic disorders up to a cross-section myelitis. Are frequent at a lymphogranulomatosis of change of a skin. Scratches, allergic implications, an anhidrosis.

The fever at a lymphogranulomatosis is diverse. At subacute flow and in end-stages is often observed wavy rise in temperature with gradually shortened intervals between waves. The sweating becomes perceptible all patients. A dermal itch - at 25-35 % of patients. Expression is various (from moderated in the field of the enlarged lymphonoduses to a generalised dermatitis with scratches. Specific to a lymphogranulomatosis of changes of peripheric blood is not present. At 1/2 patients - moderated нейтрофильный a leukocytosis. At serotinal stages - a lymphocytopenia. The anaemia, a leukopenia, a thrombocytopenia - are frequent at serotinal stages. The ESR augmentation - associates with rising of the maintenance of a hepatocuprein, haptoglobin, a fibrinogen. The myelogram has no essential deflections.

Clinical classification

• Stage 1. A lesion of lymphonoduses of one range (I) or a lesion of one out of a lymphatic organ or localisation (IЕ)
• Stage 2. A lesion of lymph nodes of two ranges and more on one party of a diaphragm (I I) or the same and localised lesion of one out of a lymphatic organ or localisation (I IЕ) on the same party of a diaphragm.
• Stage 3. A lesion of lymph nodes of any ranges on both parties of a diaphragm (I I I), accompanied or the localised lesion of one out of a lymphatic organ or localisation (I I IЕ), or a lien lesion (I I IS) or a lesion and that and another (I I IЕS).
• Stage 4. A diffusive lesion of one or more organs with a lesion of lymphonoduses or without them.

Lymphogranulomatosis flow is diverse - from benign, tightened on many years, to the subacute, leading patient of mors for some months.

Worse: at men, at children and elderly; pregnancy, an insolation, phiz procedures unfavorablely influence L flow.

Precursory symptoms of unfavorable flow: the ESR more than 30 mm/ch, rising of concentration of a fibrinogen more than 5.0 g/l, a2-globulin more than 10 g/l, a haptoglobin more than 1.5 mg of % is enlarged, a hepatocuprein more than 0.4 units Are so-called "biological" indicators of activity.

The lymphogranulomatosis can become complicated an acute asphyxia, a prelum of a cholic duct> an icterus, development of intestinal impassability. The most terrible complication - an amyloidosis of nephroses and an intestine. A cause of death - disease advance> a cachexia, is pulmonary-warm, hepatic, hepatonephric insufficiency, an amyloidosis.

Immune defect in the form of sharp oppression or abaissement dermal reaction of the slowed down type is inherent in a lymphogranulomatosis. But ability to antibody formation remains. I.e. defect is bound to function disturbance leukoferon T-lf. The augmentation of number of a T suppressor> an immunologic hyporeactivity Is revealed. The antibodies fixed on leukoferon T-lf and depressing their activity are besides, taped. Immunologic at a lymphogranulomatosis are characterised by larger predisposition of patients to a tuberculosis, virus diseases. At cytogenetic researches of a stuff of the struck lymphonoduses more than at half of patients karyotype disturbances - lines of abnormal polyploid cells are taped.

Diagnostics now the morphological. The morphological diagnosis is considered authentic at detection Sternberg cell. But Sternberg cell not pathognomonic for a lymphogranulomatosis. Set of morphological data is necessary. Sometimes reception of a stuff for histological research is complicated by locus localisation in lymphonoduses of a mediastinum or retroperitoneal space. Apply a mediastinoscopy with a biopsy or a diagnostic thoracotomy. Localisation in retroperitoneal space - the indication for a diagnostic laparotomy. Application of a computer tomography for definition of diffusion of process is possible. To research of periaortal lymphonoduses apply a lymphography. Radiological revealing of process in bones - the certificate of far come lesion. Radioisotope research with a technecium and strontium defines an osteal pathology much earlier. Absolute criterion of a lesion of KM and liver is data of histological research (the KM trepanobiopsy, a liver puncture biopsy).

Treatment. 1. Radial therapy - in case of a local lesion - an irradiating of the locuses. Treatment - to 90 %

2. Chemotherapy. Application of vinblastine, Natulanum, Chlorbutinum as monotherapies is used was rarely. Apply a complex of preparations, sometimes together with radial therapy. The best combination of preparations - EVNP (Embichinum, vinblastine, Natulanum, Prednisolonum). Gives 55-81 % of full remissions.