Pediatric Hemangioma Thrombocytopenia Syndrome

Introduction to pediatric hemangioma thrombocytopenia syndrome Hemangioma thrombocytopenia syndrome (Capillaryangioma-thrombocytopeniasyndrome), also known as giant hemangiomatosis, Kasabach-Merritt syndrome, is a congenital disease, and patients have giant cavernous hemangioma , thrombocytopenia and purpura, etc., more common in the neonatal period. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: anemia

Pediatric hemangioma thrombocytopenia syndrome etiology

(1) Causes of the disease

Mostly congenital vascular malformations, cavernous hemangioma is formed by the extension of most vascular tissue, saclike expansion into a mass, named because of its soft and sponge-like, if the hemangioma is small, the range is not large, not a sponge It is not easy to cause thrombocytopenia.

(two) pathogenesis

The pathogenesis of this disease is intravascular coagulation in the tumor. It is also possible that platelets are used as the endothelium of hemangioma and the cause of thrombocytopenia. It is also believed that the reticular effect of the reticuloendothelial system is enhanced, and hemangioma may produce platelet antibodies. Platelets have a destructive effect, and the blood vessels in the hemangioma are abnormal, causing platelet aggregation to stagnate in the tortuous hemangioma, platelet injury and lysis.

Pediatric hemangioma thrombocytopenia prevention

At present, there are no effective preventive measures, and it is mainly timely to find timely treatment.

Pediatric hemangioma thrombocytopenia syndrome complications Complications anemia jaundice

Compression symptoms of adjacent organs and tissues can occur; skin purpura and bleeding occur. Anemia and jaundice can occur.

Pediatric hemangioma thrombocytopenia syndrome symptoms common symptoms skin purpura spot nasal bleeding hepatosplenomegaly thrombocytopenia skin mucosal bleeding jaundice intravascular coagulation

When the hemangioma is on the surface of the body, it can be found after birth, but it is not easy to find in the visceral or internal tissues. The hemangioma can be large, vascular endothelium, capillary type, cavernous sinus, etc. Progression and increasing, local blue-purple, soft, cystic, compressible, hemangioma single or multiple, the most common in the limbs, usually benign hemangioma, large hemangioma distributed in the internal organs and deep tissues Compression symptoms of adjacent organs and tissues can occur. The growth of hemangioma is directly proportional to the decrease of platelets, purpura and hemorrhage, but the spleen often does not enlarge.

Examination of hemangioma thrombocytopenia syndrome in children

1. Blood routine: thrombocytopenia, often (10 ~ 40) × 10 ⁹ / L. Generally, hemoglobin and white blood cells are normal, and microvascular and hemolysis may be caused by hemoglobin reduction. Blood smears may show abnormal red blood cells and more broken red blood cells.

2. Prolonged bleeding time, poor blood clot retraction, prolonged prothrombin time.

3. Decreased fibrinogen; increased fibrin degradation (FDP); positive for 3P test.

4. Bone marrow: Normal or increased megakaryocytes, CT, B-ultrasound or magnetic resonance can detect visceral lesions; beam arm test is positive.

Diagnosis and diagnosis of hemangioma thrombocytopenia in children

diagnosis

1. General performance: More common in infants and young children, usually within 5 weeks after birth, hemangioma is more common in the limbs, multiple, and secondly in the face, neck, trunk, etc., about 10% occur in the internal organs such as the liver, Spleen, ileum, tongue, kidney, chest, bone, extracorporeal, internal carotid artery, etc., accompanied by corresponding symptoms and signs, surface hemangioma pressure can be reduced, can be restored after losing pressure; liver and spleen Aneurysms can cause hepatosplenomegaly. Most of the cerebellum are malignant, and the subcutaneous and visceral lesions of the same patient rarely exist at the same time.

2. Bleeding: Mainly skin mucosal bleeding points, ecchymosis or purpura. Hemorrhagic symptoms may also occur in other parts of the nose. Hemorrhagic symptoms may increase with the development of hemangiomas. Anemia and jaundice may occur due to microvascular hemolysis. The presence of hemangiomas at birth or soon after birth is accompanied by platelets. Reduced, chronic diffuse intravascular coagulation test changes, easy to diagnose; but sometimes hemangioma occurs in the internal organs, such as the chest, liver, spleen, bones, etc. are neglected, if the blood fibrinolytics (FDP) increase more helpful For diagnosis.

Differential diagnosis

The disease is mainly differentiated from disseminated intravascular coagulation (DIC), DIC disease progresses rapidly, and there are many serious infections, shock, vital organ failure and microvascular hemolysis.

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