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Osler's disease

Osler's disease — one of forms of myeloses with defeat of a cell predecessor of a myelopoiesis and its unlimited proliferation, preservation of its differentiation potency on 4 sprouts, mainly erythroidal.

Epidemiology
The Osler's disease on structure and annual average indicators of incidence of leukoses takes the 4th place after a myelosis. Family cases of an Osler's disease are observed. People get sick mainly 40 years, men are more senior a little more often than women.

Etiology and pathogeny
(see Hemoblastoses)
The clonal nature of a miyeloproliferation is established by the researches G-6-FD at patients with an Osler's disease of mulattos, heterozygous on this enzyme: in erythrocytes, granulocytes and thrombocytes only one type of this enzyme while in fibroblasts of marrow and skin both its types are found is revealed. The clonal origin of cellular substrate of an illness is proved by also cytologic researches which established disturbances of a karyotype in groups of chromosomes With, CD and G. Defects of chromosomes (a psevdoploidiya, an aneuploidy, structural aberrations) do not meet in lymphocytes. The role of increase in a pool of eritropoetinchuvstvitelny cells in a pathogeny is not excluded.

By radiological researches it is shown that at an Osler's disease the erythrogenesis is sharply strengthened, its normal topography is revealed. The erythrogenesis gradually decreases in flat bones, and with development of a myelofibrosis and osteosclerosis — and in tubular.

Classification
The initial (I) stage of an Osler's disease is characterized by the minimum clinical and hematologic manifestations; ON and stage NV — eritremichesky — with the developed process picture, absence or existence of a myeloid metaplasia of a spleen; The III stage — anemic — can be combined with a depression trombo-and a leukopoiesis, in this stage are observed a plethora, spleno-and a hepatomegalia with their myeloid metaplasia. Outcomes in an acute leukosis, a myelosis, a hemopoiesis hypoplasia, a myelofibrosis are possible.

Approximate formulation of the diagnosis:
1. An Osler's disease with a little increased quantity of erythrocytes, thrombocytes and leukocytes, with insignificant increase in a spleen (an initial stage).
2. An Osler's disease with the expressed hyperglobulia, a thrombocytosis and a leukocytosis, increase in a spleen and liver, fibrinferments (an eritremichesky stage).
3. The Osler's disease proceeding with anemia, a depression trombo-and a leukopoiesis, progressing spleno-and a hepatomegalia (an anemic stage).

Clinic
The Osler's disease is characterized by long and rather high-quality current. Face skin, ears, a tip of a nose, distal departments of extremities and visible mucous patients have red and cyanochroic coloring of various degree of manifestation. Frequent complaints are the headache, dizziness, fatigue, sleeplessness, feeling of "weight" in the head, a sonitus, numbness and tingling in fingers, sometimes vision disorders. Research of an eyeground reveals congestive gyrose vessels, a papilledema, in rare instances a retinal embolism. At patients paralyzes, epileptoidny attacks, the progressing decrease in memory and working capacity, a depression can be observed. Seldom the ulcer of a duodenum or stomach, bleeding from varicose expanded veins of a gullet, a stomach, intestines, fibrinferment of mezenterialny vessels meet.

The Osler's disease can be suspected at men with quantity of erythrocytes more than 5,7*109/l, hemoglobin more than 177 g/l and a hematocrit more than 52%, and at women with quantity of erythrocytes more than 5,2*109/l, hemoglobin more than 172 g/l and a hematocrit of 48 — 50%. With process progressing these indicators usually increase, moderately expressed thrombocytosis and a leukocytosis with emergence in blood of single metamyelocytes and myelocytes joins. Viscosity of blood increases by 5 — 8 times, SOE is slowed sharply down. Longevity of erythrocytes normal or shortened, sometimes is defined their raised sequestration in a spleen. The mass of the circulating erythrocytes is increased.

Punctate of marrow is often diluted with peripheral blood therefore it is more reasonable to investigate tissue specimens. Reduction of maintenance of fatty marrow, a hyperplasia of three sprouts of a myelopoiesis, quantity eritro-is characteristic of an Osler's disease and normoblasts can sometimes be moderately raised.

The Osler's disease in a stage of a myelofibrosis is followed by spontaneous normalization of indicators of hemoglobin and erythrocytes, increase of number of leukocytes with shift to unripe forms, emergence of erythroblasts in a gemogramma, fast increase in the sizes of a spleen and liver.

Verification of the diagnosis
Diagnosis of an Osler's disease is based on data gemogramm, conditions of a marrowy hemopoiesis and clinical symptomatology. Differential diagnosis is carried out with the secondary hyperglobulias which are found at a hypoxia (inborn "blue" heart diseases, a pneumosclerosis, adaptation to highlands conditions), some tumors (kidneys, a cerebellum, etc.), pathology of kidneys (local renal ischemia, a hydronephrosis, a polycystosis, a stenosis of renal arteries, etc.), thrombosis of hepatic veins (Badd's syndrome — Kiari), liver cirrhoses, visceral tuberculosis, etc.

The Osler's disease should be differentiated from the hereditary hyperglobulias (a family polycythemia, primary hyperglobulia) more often having family character. They meet in various geographical areas, in our country the disease centers with an autosomal and recessive mode of inheritance are for the first time revealed among inhabitants of Chuvashia. The main differential and diagnostic criteria of an Osler's disease and heredo-familial hyperglobulia are provided in the table.

Kliniko-gematologichesky signs of an Osler's disease and heredo-familial hyperglobulia

Symptoms

Osler's disease

Heredo-familial
hyperglobulias

Hyperglobulia

In 93% of cases

In 100% of cases

Quantity of leukocytes

It is increased in 45 — 65% of cases

Normal or is reduced

Neutrocytosis

In 75 — 80% of cases

No

Histologic picture

Trekhrostkovy hyperplasia

Moderate hyperplasia at the expense of mature forms of an erythrogenesis, relative reduction of quantity of cells of a megakariotsitopoez

Liver

It is increased in 40 — 68% of cases

Insignificant passing increase in 34% of cases

Spleen

The progressing increase in 70 — 78% of cases

Insignificant passing increase in 12% of cases

Arterial pressure

Increase in 32 — 35% of cases

Decrease in 73% of cases

Symptoms

Osler's disease

Heredo-familial hyperglobulias

Ostealgias

In 30% of cases

No

Skin itch

In 43 — 64% of cases

No

Age of patients

80% of cases are more senior than 40 years

72% of patients — children and young men

 

At secondary and hereditary hyperglobulias ability to proliferation of elements of an erythroidal sprout of marrow is considerably increased. It is noted that at hyperglobulias the quantity of PAS positive erythroidal elements are more, than at an Osler's disease.

At family hyperglobulias the IDCS caused by the excess content of thromboplastic substances in erythrocytes can develop. Thus the hypercoagulation which is followed by thrombosis comes to light more often, hemorrhages meet less often.

Treatment
In an initial stage of the Osler's disease proceeding only with increase in a hemoglobin content and erythrocytes 2 — 3 bloodlettings are applied usually (on 500 ml of blood each 3 — 5 days with the subsequent introduction sick adequate quantities of a reopoliglyukin or isotonic solution of sodium chloride). At high rates of the ABP and atherosclerosis the volume of bloodletting makes daily 200 — 250 ml before decrease in a hematocrit and number of erythrocytes in peripheral blood. Patients with initially high thrombocytosis in the conditions of bloodletting can have an increased risk of development of trombotichesky complications.

In recent years with success apply eritrotsitaferez, allowing to delete sick 1000 — 1200 ml of erythrocytes from an organism, plasma with dezagregatsionny means reopoliglyukiny returns to a blood channel, unlike bloodletting the cytapheresis does not cause a hypoproteinemia, decrease in volume of the circulating blood and hypercoagulation.

The indication for purpose of cytostatic therapy is the developed disease stage.
Myelosanum apply inside on 4 — 6 mg a day (a course dose on average 300 mg). Drug is cancelled when the number of leukocytes and thrombocytes decreases to 4*109/l and 100*109/l respectively. The course of treatment usually lasts 2 — 2,5 months. At sharply expressed plethora reception of Myelosanum is combined with bloodlettings or eritrotsitaferezy. The supporting treatment with Myelosanum in doses of 2 — 4 mg a week is begun not earlier than in 3 months after the termination of a basic course of therapy when the possibility of cumulative action of drug is excluded.

When using Imiphosum (intramusculary on 50 mg every other day, an average course dose of 500 — 600 mg) more often than at purpose of other cytostatic drugs, there are cytopenias, especially thrombocytopenia therefore in the presence of it it is not necessary to apply this means. Gemogramma is investigated before each administration of drug. Appoint Imiphosum sick with a high megakariotsitoz and a thrombocytosis, with considerably the increased spleen. Its depressive influence on a marrowy hemopoiesis is observed during 6 weeks and more after the end of treatment.

Miyelobromol apply inside 250 mg daily or every other day, the average course dose of 7500 mg, a course of treatment of 1,5 — 2 months of Gemogramm is investigated 1 time in 10 days. The cytopenia develops less than at treatment by Myelosanum and Imiphosum. In a stage of a myelofibrosis use miyelobromol or Cyclophosphanum. At development of thromboses appoint anticoagulants.

Use of a hydroxycarbamide is possible (see the Myelosis). At fibrinferments appoint anticoagulants, at hemorrhages — transfusions of a trombokontsentrat, at a hyperuricemia — Allopyrinolum.

 
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