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Differential diagnosis of a polycythemia
Hypoxemic and relative polycythemia
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The term "polycythemia" is applied by us (as well as other authors) not in a literal sense, and to designation of group of diseases which are shown by increase in mass of erythrocytes in an organism, i.e. increase in their volume by 1 kg of body weight. Clinically it is expressed in absolute increase in number of erythrocytes which is frequent, but not always, is followed by increase of concentration of hemoglobin and a share of erythrocytes in the total amount of blood.
The volume of erythrocytes in an organism of the healthy man averages 36 ml/kg, and in the woman's organism — 32 ml/kg. Saturation of an arterial blood oxygen at the healthy person does not fall lower than 92%. It is accepted to distinguish an absolute polycythemia — the independent illness of an unknown etiology proceeding with proliferation of all sprouts of marrow, and secondary, or symptomatic, a polycythemia which is shown by increase in mass of erythrocytes in an organism without the accompanying involvement in process of other sprouts of marrow. It is accepted to allocate, besides, still relative, pl false, a polycythemia at which the mass of erythrocytes in an organism does not change, but because of reduction of volume of plasma concentration of erythrocytes and hemoglobin in blood considerably increases. Thus, determining the volume of erythrocytes on 1 kg of body weight, it is easy to separate a relative polycythemia from true and symptomatic.
The clinical picture of an Osler's disease was for the first time described by Vaquez in 1892. The illness is found usually in the middle age. Men are ill approximately twice more often than women. The polycythemia almost does not occur at children. In pronounced cases the polycythemia is diagnosed without effort on set of signs: reddish cyanosis of the person and extremities, ecchymomas on skin, sharply expressed network of expanded blood vessels of conjunctivas and scleras of eyes poured by blood visible mucous membranes, a considerable splenomegaly, hyperplastic marrow, the increased number of erythrocytes, leukocytes and platelets, the increased concentration of hemoglobin in blood and the increased hematocrit size. The majority of the listed symptoms and signs is explained by increase in total amount of blood, increase of its viscosity and delay of a blood-groove in the vessels overflowed with erythrocytes, especially capillaries and veins.
In most cases the illness begins imperceptibly with lowering of endurance to usual operational and household loads. Later short wind, dizziness, headaches, vision disorders join it. Sometimes there is an itch of all body with characteristic strengthening of intensity after reception of a hot bathtub. Some patients show complaints to burning or throbbing pains in feet, shins and brushes. Skin of brushes and feet becomes dark red. Sometimes Reynaud's syndrome develops.
Paradoxical tendency of patients with a polycythemia at the same time both to fibrinferments, and to the raised bleeding is characteristic. The purpura is one of characteristic symptoms of this illness, approximately stomach ulcer or a duodenum which can be complicated by profuse gastrointestinal bleeding is found in 10 — 19% of patients. At the women suffering from a polycythemia uterine bleedings which sometimes happen so plentiful that at patients the iron deficiency anemia develops are quite often observed. The small wounds remaining, for example, after extraction of tooth quite often become complicated at these patients with massive bleeding.
At the same time with tendency to bleeding at patients with a polycythemia the expressed tendency to intravascular fibrinferments is noted. Fibrinferments of small vessels of brushes and feet which sometimes take for thrombophlebitises are especially often observed. Fibrinferments of larger arterial and venous vessels are observed with special constancy in heart, a brain, a liver, a spleen and lungs. Sometimes thrombosis of a coronary or brain artery appears the first disturbance forcing the patient or his relatives to see a doctor who opens an actual reason of a stroke, myocardial infarction or acute thrombosis of a portal vein.
Change of the sizes of a spleen at a polycythemia has very big diagnostic and not less important predictive value. By the time of establishment of the diagnosis approximately it is possible to find increase in a spleen in 75% of patients. Its rounded dense edge acts from under a costal arch usually on 1 — 2 cm. Over time the spleen increases. The more the spleen size, the is more probable development than the centers of a myeloid metaplasia in it; the more illness duration, the more often is found increase in a liver. Moderate or substantial increase of arterial pressure with the accompanying hypertrophy of a left ventricle of heart is found approximately in a half of patients with the expressed illness picture.
Results of laboratory researches have great diagnostic value. The most characteristic symptom of an illness is the hyperglobulia. The number of erythrocytes in 1 ml of blood at a polycythemia raises to 7 — 10 million, and a hemoglobin content — To 180 — 240 g/l. Depending on weight of an illness the indicator of a hematocrit fluctuates from 55/45 to 70/30. As it was already noted, the mass of erythrocytes at a polycythemia sharply increases whereas the volume of plasma can be normal, slightly increased or even reduced. If the patient transferred a hemorrhage recently, the hemoglobin content and erythrocytes in blood can sharply go down. After massive bleedings even the short-term iron deficiency anemia sometimes develops.
The polycythemia proceeds usually with a moderate leukocytosis and a thrombocytosis. At 50 — 70% of patients with a polycythemia without symptoms of any infection the maintenance of leukocytes in blood is increased usually to 10*103 — 12*103 in 1 mkl. Occasionally also higher leukocytosis meets. Increase in number of leukocytes is explained by their faster development in marrow. Natural increase in number of thrombocytes explains special tendency of patients with a polycythemia to formation of vascular blood clots.
In punctates of marrow of patients with a polycythemia symptoms of a hyperplasia of usually erythrocyte and megakariotsitarny sprouts are found. In blood serum of patients with a polycythemia quite often it is found, as well as at other myeloproliferative syndromes, increase of concentration of an alkaline phosphatase, uric acid and B12 vitamin.
Before introduction of modern methods of therapy longevity of the patient with a polycythemia fluctuated from 1 to 25 flyings from the moment of detection of an illness and averaged 6,7 years. Average longevity at modern methods of treatment doubled. The chronic slowly progressing current of a polycythemia is interrupted by the acute complications caused by either fibrinferments, or hemorrhages from time to time. Thrombosis can arise in any vessel, but fibrinferments of branches of a portal vein, brain and coronal arteries which are complicated by strokes, a myocardial infarction, a portal hypertension are most often observed.
The diagnosis of an Osler's disease often is very simple. The illness is supposed by outward of the patient. This assumption becomes reasonable after determination of mass of erythrocytes. The diagnosis is taken for granted if it is not possible to find the diseases capable to be complicated by a symptomatic (secondary) polycythemia in the patient. Our experience shows that the Osler's disease quite often remains for a long time not distinguished. More often we had to distinguish it from an idiopathic hypertensia, cirrhosis, leukoses, renal colic.
The polycythemia at early stages of development quite often is accepted to an idiopathic hypertensia. The mass of erythrocytes in this period of an illness is increased still slightly, and in outward of the patient there were no characteristic signs excessive krovenapolneninya skin, an eye, mucous membranes yet. Average age of the patient and the complaint to memory easing, headaches, dizziness, a sonitus, an asthma and pains in heart allow to think first of all of more often found atherosclerosis of coronal and brain arteries. Increase of arterial pressure quite explains both headaches, and a sonitus, and, perhaps, a little early emergence of angina pectoris. The typical for a polycythemia alternating lameness is estimated as one more display of system atherosclerosis, in this case the lower extremities. Developing of a myocardial infarction or stroke at a polycythemia is perceived sometimes as natural complication of coronary heart disease or a brain, and the heart failure developing at the same time is estimated as a result of a cardiosclerosis.
Unsuccessfulness of hypotensive therapy at the patient with a polycythemia with the increased arterial pressure quite often appears the first reason forcing to reconsider separate links in the chain of conclusions stated above. In most cases usually proceeding nonmalignant idiopathic hypertensia combined action of a bed rest and modern antihypertensives is followed by normalization of arterial pressure and disappearance of subjective displays of an illness. The same therapy in cases of a polycythemia can lead to normalization of arterial pressure, but the subjective picture of an illness at the same time remains without changes. Patients still note attacks of angina pectoris, a sonitus, headaches.
The number of diagnostic mistakes would considerably be reduced if in each case of an arterial hypertension comparison of a clinical picture of an illness to a condition of an eyeground was surely made. Eyeground vessels at a polycythemia with the increased arterial pressure are always overflowed with blood, and the blood stream in them is always slowed down. Such picture of an eyeground is never observed at an idiopathic hypertensia of which the spastic condition of arteries and the normal or lowered krovenapolneniye of capillaries are characteristic.
The increased maintenance of leukocytes and thrombocytes in blood is a characteristic sign of a polycythemia. The leukocytosis is observed only in the cases of an idiopathic hypertensia Complicated by an infection. The increased maintenance of thrombocytes in blood, the purpura and extraordinary bleeding from small wounds are characteristic of a polycythemia and do not meet at an idiopathic hypertensia.
Combination of the increased arterial pressure to the increased spleen — a cardinal sign of a polycythemia. In all cases when this sign comes to light, it is necessary to think of a polycythemia and to determine the mass of erythrocytes in an organism. Most precisely she manages to be defined using radioactive chrome. The diagnosis of a polycythemia can be considered fixed if at the patient increase in a spleen and mass of erythrocytes is revealed exceeds the normal amounts stated above.
In cases of far come polycythemia with the increased arterial pressure, an asthma, headaches, angina pectoris and characteristic outward of patients quite often there are attacks of acute pains in left hypochondrium which usually are caused by spleen heart attacks. Sometimes these pains are accepted to left-side renal colic. The actual reason of suffering can be found out usually from the patient's bed without additional researches. Pains at a heart attack of a spleen either remain local, or irradiate up to the area of the left shoulder whereas pains at left-side renal colic irradiate down to the left inguinal area and in the left small egg. Pains at a heart attack of a spleen amplify at breath, when pressing on area of the left hypochondrium. Pains at renal colic do not depend on breath. The friction murmur of a peritoneum is sometimes listened over a spleen at her heart attack, he never is neither at renal colic, nor at a heart attack of kidneys. The characteristic uric syndrome naturally appears at renal colic and at a heart attack of kidneys. The analysis of urine at a heart attack of a spleen remains normal. Crucial importance nevertheless should be attached to identification of symptoms of the main illness.
The expressed increase of viscosity of blood in far come cases of a polycythemia is complicated by fibrinferments of small and larger vessels. Blood clots with special pattern are formed in vessels with the slowed-down current of blood. Especially frequent formation of thromboses in splenic branches of a portal vein is also explained by it. The arising portal hypertension increases stagnation of blood in the pool of a portal vein and promotes development of a porto-caval anastomosis and ascites. The clinical picture of an illness in similar cases reminds a cirrhosis picture.
The correct diagnosis of the reason of ascites and hepatolienal syndrome in the majority of similar cases manages to be put at the patient's bed.
The polycythemia with ascites, increase in a liver and spleen differs from true cirrhoses of a liver in expressiveness of the accompanying symptoms of an illness. Arterial pressure at patients with a polycythemia is quite often put by raised. In all cases observed by us noted the normal or increased maintenance of erythrocytes and thrombocytes in blood, complaints to headaches, dizzinesses, a sonitus, attacks of angina pectoris, the alternating lameness, short wind. Quite often on an ECG of these patients symptoms of the myocardial infarction postponed earlier come to light. Content of B12 vitamin and an alkaline phosphatase in blood increases.
The ascitic stage of cirrhosis proceeds with exhaustion of the patient, arterial pressure at it is normal or lowered, the content of proteins in blood plasma is reduced, anemia quite often is defined. Muscles of an upper half of a trunk are atrophied, and on skin vascular stars points often are found.
The final diagnosis can be confirmed with results of a puncture biopsy of a liver. The liver structure at a polycythemia remains normal whereas at cirrhosis data of punctate allow to speak about disturbance of normal architecture of hepatic segments, and is frequent also about existence of signs of an inflammation in the connecting fabric surrounding them. The compared diseases differ from each other also according to a stsintigrafiya of a liver and a portografiya, but need for carrying out these researches usually does not arise.
Later 10 — 20 after the beginning of a polycythemia in peripheral blood of patients unripe cells of a miyelopdny row appear. They differ from unripe cells at a myelosis on the maintenance of an alkaline phosphatase, glycogen and histamine. In similar cases it is accepted to speak about transformation of a polycythemia in a myeloleukemia or a myelofibrosis. The expressed polycythemia cases differ from a myeloid leukosis markedly, but it cannot be told about their initial manifestations when both illnesses can proceed with moderate increase in a spleen, a hyperplasia of all sprouts of marrow and unsharp increase in mass of erythrocytes.
Difficulties of the differential diagnosis in similar cases sometimes manage to be overcome a ratio of an onset of the illness and age of the patient. The polycythemia begins, as a rule, in the middle age, and leukoses quite often occur at young people. The specified rule is not absolute, and it cannot be put to the analysis of an individual case of an illness.
The final diagnosis manages to be established usually only after more or less long supervision over disease. The patient with a polycythemia has a characteristic outward caused by a hyper hyperglobulia over time, the number of platelets increases, hematocrit figures accrue, angina pectoris develops. Progressing of a myeloleukemia is followed by emaciation, development of usually moderate fever, perspiration at night, anemia which sometimes has a hemolitic origin. The differential diagnosis is carried out in most cases, without resorting to results of a sternal puncture.
The maintenance of erythrocytes in blood can is long to increase under the influence of many factors; among them especially large role is played by some hormones, content of oxygen in blood, structural features of a structure of a molecule of hemoglobin, etc. The secondary polycythemias arising at these disturbances according to the reasons which caused them can be subdivided into 3 groups, the smallest of which is recently described hereditary polycythemia, or primary hyperglobulia, and the most widespread — group secondary polycythemias, the fabrics arising in connection with a hypoxia. The polycythemias developing sometimes at tumors are allocated by us in separate group because of an ambiguity of mechanisms of their development.
The inborn polycythemia is studied mainly on children. Autosomal dominant and recessive forms of an illness are described. Mechanisms of development of a polycythemia remain still insufficiently found out. The illness is shown as selective increase in number of erythrocytes. The maintenance of leukocytes and thrombocytes in blood remains without changes. The spleen moderately increases in sizes. The illness begins in the childhood, proceeds is good-quality, usually with moderate increase in a spleen. Tromboembolic episodes occur at patients with an inborn polycythemia nevertheless a little more often than at healthy people.