DISEASES OF THE BLOOD SYSTEM : VERLOOVE DISEASE (MORBUS MACULOSUS WERLHOFH) (CHRONIC RECURRENT PURPURA) - etiology, pathogenesis, symptoms and course of, recognition, prediction and prevention
The etiology is unknown. Particular importance should be given to infection and trauma as precipitating bleeding factors.
The pathogenesis. Crucial defeat of the vascular wall endothelium of capillaries and trombopenia contributing to the onset of bleeding.
Symptoms. Complaints about bleeding gums, nasal and uterine bleeding, bruising. Objective examination on the skin of the trunk and limbs celebrate purple and petechiae, expressed in varying degrees. Hemorrhagic syndrome characterized by prolonged bleeding time, trombopenia, lack of blood clot retraction. Positive symptom Konchalovsky (precipitation of petechiae and purpura after pulling a rubber tourniquet upper limb), the appearance of petechiae in a day after multiple injections made by the pin on the skin of the patient. Enough sometimes slightly pinch the patient, and the skin immediately appears bruise. Anemia hypochromic type. Immediately after blood loss marked neutrophilic leukocytosis.
For. Clinically downstream distinguish two forms:
1) chronic, relapsing purple.
At first relapses are followed by remission, sometimes there is subfebrile temperature. Subacute is actually a chronic form, as would converting it into a continuous with progressively more sophisticated phenomena hemorrhagic diathesis - temperature reaction in the form of a severe fever.
Recognition - based on the above is no problem. You must differentsirovat with anemia of various types, with acute leukemia and scurvy.
Prediction of chronic recurrent purple relatively favorable, subacute bad.
Splenectomy. It should be noted that this operation is a serious and very responsible, so evidence must be strictly balanced. Full cure under the effect of this operation is not observed; after a given period of time phenomena characteristic of hemorrhagic diateza, reappear, but in most cases do not reach the level observed before the surgery. Of course shown to operations subacute cases, where there is hyporegenerative type of anemia, if gipoplasticheskaya type of anemia operation is hopeless. Chronic recurrent purple, in the presence of persistently occurring relapses (progression of hemorrhagic diathesis, durability anemia), it is appropriate to offer the operation. To assess the indications for splenectomy should be considered not only with painting punctate bone marrow, but also with the patients ' age and concomitant diseases. Transfusion of blood at a dose of 200 - 300 ml resend or transfusion of concentrated mass of erythrocytes in the same dosage, in some cases, cause a remission duration of several months. You should also use plasma and serum (injected dose of 30 ml re). Transfusion of erythrocyte mass should be widely used in those cases when it is supposed to remove the spleen.