cavernous hemangioma thrombocytopenia syndrome

Introduction

Introduction to cavernous hemangioma thrombocytopenia syndrome The cavernous hemangioma thrombocytopenia syndrome was first reported by Kasabach-Merritt in 1940. basic knowledge The proportion of illness: 0.015%--0.03% Susceptible people: no specific population Mode of infection: non-infectious Complications: nosebleeds

Cause

Cavernous hemangioma thrombocytopenia syndrome

Causes:

Currently no relevant information

Pathogenesis

Most of the pathological changes of this disease are congenital. The lesions are gradually obvious in adolescence or adulthood. Cavernous hemangioma is caused by the extension of most vascular tissues, and it is caused by a cluster. If the hemangioma is small, the range is small. , it does not necessarily cause thrombocytopenia, therefore, it is generally necessary for a giant cavernous hemangioma to cause thrombocytopenia.

After the platelets are introduced into the patient, they disappear quickly and the survival time is shortened by half. The platelets are labeled with radionuclides and scanned in vitro. The radioactivity is concentrated in the hemangioma, and the platelet thrombus is seen in the biopsy at the lesion.

The cause of thrombocytopenia is caused by increased platelet consumption, platelet retention in hemangioma, intravascular coagulation of hemangiomas, and even chronic intravascular coagulation, resulting in increased platelet consumption and thrombocytopenia. Patients often have fibrinogen. Factor II, factor V, factor VII reduction, accompanied by secondary fibrinolysis, plasma protamine paracoagulation experiments were positive.

Prevention

Cavernous hemangioma thrombocytopenia syndrome prevention

1, mostly based on light food, pay attention to the law of diet.

2. Eat properly according to the doctor's advice.

3, the disease does not have too much taboo on the diet, a reasonable diet can be.

Complication

Cavernous hemangioma thrombocytopenia syndrome complications Complications, nosebleeds

Skin spots, ecchymosis, nosebleeds, oral bleeding.

Symptom

Cavernous hemangioma thrombocytopenia syndrome symptoms common symptoms thrombocytopenia nasal bleeding lip bleeding skin purpura

Spongiform hemangioma of the skin can occur in the face, arms, trunk and neck. 30% occurs in the limbs and is multiple. It occurs in the viscera, accounting for about 10%. It is often not obvious at birth. As the age increases, the lesions increase gradually. Big.

The main symptoms of bleeding are skin stasis, ecchymosis, nosebleeds, oral bleeding, and a small number of deaths due to bleeding.

Examine

Examination of cavernous hemangioma thrombocytopenia syndrome

1. Peripheral blood: hemoglobin, red blood cells, white blood cells are normal, platelets are often reduced (10 ~ 40) × 109 / L, bleeding time is prolonged, blood clot retraction is poor, red blood cell damage of microvascular hemolytic anemia can be seen in peripheral blood smears Such as malformed red blood cells, broken red blood cells, red blood cell debris, helmet-type red blood cells and so on.

2. Bone marrow: megakaryocytes are normal or increased.

3. Fibrinogen: Reduced sometimes severely decreased in concurrent DIC, prolonged prothrombin time, positive for FDP, positive for plasma protamine coagulation test.

According to the condition, the clinical performance is selected to do B-ultrasound, X-ray, electrocardiogram and other examinations.

Diagnosis

Diagnosis and diagnosis of cavernous hemangioma thrombocytopenia syndrome

Diagnosis can be based on clinical manifestations and thrombocytopenia.

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