Crescently - cellular anemia

Description:

The sickemia to belong to qualitative hemoglobinopathies, eurysynusic at inhabitants of tropical Africa, less often it occurs at inhabitants of the Mediterranean, the Middle East, in South America. It is important to note that it is widespread preferential in regions, unsuccessful on malaria.

Sickemia symptoms:

Drepanocytic anemia is clinically characterized by the symptoms caused, on the one hand, by thrombosis of vessels of various bodies crescent erythrocytes, and with another - hemolitic anemia. Severity of anemia depends on concentration of HbS in an erythrocyte: the it is more, the symptomatology is brighter and heavier. Besides, at erythrocytes there can be also other pathological haemo globins: HbF, HbD, NS, etc. Sometimes drepanocytic anemia is combined with a thalassemia, at the same time clinical manifestations can decrease or, on the contrary, accrue.

In the initial period of a disease preferential marrowy system is surprised: there is a swelling, and also pain at the expense of thrombosis of the vessels feeding a joint and a bone. The aseptic necrosis of a head of a femur with accession of an infection and osteomyelitis is possible further. Hemolitic crises develop usually after the postponed infections, have regenerator or hyporegenerative character and are the main reason for death of these patients. Sekvestratsionny crisis due to deposition of blood in a spleen and a liver which is expressed by a pain abdominal syndrome due to bystry increase in these bodies is in rare instances observed and is followed by a collapse; at the same time hemolysis can be absent, meets pulmonary heart attacks in connection with disturbance of microcirculation at the level of pulmonary vessels.

In the second period a constant symptom - hemolitic anemia. The marrow hyperplasia developing in tubular bones (in them an active hemopoiesis as compensatory reaction to hemolysis is made) is followed by characteristic changes of a skeleton: thin extremities, the bent backbone, a tower skull with cambers in a forehead and a parietal bone. Gepato-and a splenomegaly develop due to activation in them of an erythrogenesis, and also secondary hemochromatosis and thrombosis; at a part of patients cholelithiasis forms. The hemosiderosis of a cardiac muscle leads to heart failure, and a hemosiderosis of a liver, a pancreas - to cirrhosis and a diabetes mellitus.

Thrombosis of vessels of kidneys proceeds with a hamaturia and the subsequent renal failure. The neurologic symptomatology is caused by a stroke, paralysis of cranial nerves, etc. Trophic lower extremity ulcers are characteristic. Most of patients with a severe form of drepanocytic anemia perishes within 5 years, and endured this term enter the third period which is characterized by symptoms of unsharply expressed hemolitic anemia. The spleen at them usually is not probed as repeated heart attacks lead to its wrinkling - an autosplenektomiya. The liver remains increased, unevenly condensed, and frequent infections accept quite often septic current.

Hematologic changes. Concentration of hemoglobin decreases (< 80 г л и в среднем составляет 50 г л особенно во время гемолитического криза. a target="_blank" href="index-392.htm" tppabs="medicalmeds.eu/gematologiya/anemia/">Anemia normokhromny, regenerator; a reticulocytosis - 5-15%. Erythrocytes meet Joly's little bodies. The quantity of leukocytes in the period of crisis is increased to 20×109/л. In marrow the hyperplasia of an erythroidal sprout is observed. For detection of crescent form of erythrocytes carry out special test: the drop of blood is covered with glass, pressurized for what edges of glass grease with vaseline; in a few minutes the partial pressure of oxygen in a blood drop under glass decreases and erythrocytes take the crescent form. The hemoglobin electrophoresis is more informative: at drepanocytic anemia at homozygotes bulk is made by HbS, HbA is absent, the maintenance of HbF is increased; at heterozygotes at an electrophoresis along with HbS reveal NYA. In blood the maintenance of free fraction of bilirubin is increased, the content of serumal iron is increased; osmotic resistance of erythrocytes is increased.

Heterozygous patients feel almost healthy; anemia and morphological changes of erythrocytes are found in them only in the conditions of a hypoxia (rise to mountains, the heavy exercise stress, weeds by airplanes, etc.). However hemolitic crisis and at them can end letalno.

Thus, the clinic of drepanocytic anemia is characterized by a polisimptomnost: yellowness of integuments, hypoxemic syndrome, gepatosplenomegaliya, deformation of a skeleton, repeated thrombosis of bodies; from hematologic symptoms: anemia of regenerator character, the crescent form of erythrocytes revealed at special tests, a hyperbilirubinemia at the expense of free fraction. Belonging of the person to a certain ethnic group gives the grounds to suspect this disease and to begin purposeful inspection for confirmation or an exception of this anemia.

Sickemia reasons:

Qualitative changes of hemoglobin consist that in situation ß chain glutaminic acid is replaced with valine. Pathological hemoglobin received the name from the word "sicsle" - a sickle because the erythrocyte gets a crescent form. We will dissolve HbS less, than normal, and in the conditions of a hypoxia drops out in a deposit. Regarding cases the asymptomatic carriage of HbS when its concentration in erythrocytes makes no more than 20-45% is observed; in usual conditions hemolysis does not develop, only special situations can provoke it, for example weeds by airplanes or scuba diving when there is a hypoxia. The pathogeny of hemolysis is difficult. Believe that in the conditions of acid medium in the molecule HbS there is a change of a negative charge on neutral as valine has neutral reaction, and glutaminic acid - acid. At the same time solubility of hemoglobin decreases, hydrophobic bonds between the valine remains are formed, and molecules of pathological HbS crystallize that leads to formation of a crescent form of an erythrocyte.

Part of the deformed erythrocytes changes is irreversible and prematurely collapses phagocytes. Circulating in blood, crescent erythrocytes stick together among themselves, especially in small vessels, forming blood clots and breaking blood circulation. The hypoxia which arose in these conditions promotes formation of new crescent erythrocytes - some kind of vicious circle of which are result стаз and thrombosis in various bodies, including in a spleen which gradually atrophies after a hypertrophy develops. Genetic defect is localized in a structural gene of ß chain where there was a dot mutation.

Treatment of the Sickemia:

Ways of treatment of a sickemia do not exist. However, the general improvement of medical care became a possibility of longer and healthy life of the children having this disease. Now the attention is paid to healthy nutrition, educational measures and the prevention of infections. It is very important to keep in touch with the doctor of primary help who is familiar with precursory symptoms of the complications arising at a sickemia.

Constant dispensary observation of small children with a sickemia is especially important to prevent early life-threatening infectious complications. Anemia can amplify as a result of an infection or sudden increase in a spleen, hospitalization of the child for treatment for an infection or pains can be required. Pregnancy and surgical interventions as well as diseases of eyes, require attention of the specialists having special experience with the patients suffering from a sickemia.