Additional methods of examination

Clinical blood analysis:

• activated partial thromboplastin time increased;

• whole blood coagulation time is raised;

• factor VIII dolling assay (VIII C) reduced;

• immunological methods show normal VIII R, AG;

• bleeding time and prothrombin time tests normal;

• carrier females have half the clotting activity (VIII C ) expected for the level of VIII R, G.

X-ray examination:

• broadening of femoral epicondyles;

• sclerosis, osteophyte and bony cists;

• atrophy of muscles.

The computer tomography scan: intracerebal hematoma.

Hemophilia b (Christinas' disease)

Hemophilia B (Christinas disease) occurs as a result of a deficiency of factor IX. Like Hemophilia A it is also X-linked recessive trail. The clinical feature similar to the hemophilia A but bleeding is usually not as severe because factor IX is more stable than factor VIII C.

Additional methods of examination

Clinical blood analysis

• activated partial thromboplastin time is raised;

• whole blood clotting time (severe cases) is raised;

• factor IX clotting assay is reduced;

• both bleeding time and prothrombin time tests are normal.

HEMOPHILIA C

Hemophilia C - may be defined as a bleeding disease caused by a deficiency of factor XI. It is inherited as a recessive trait. The symptoms and signs are similar to other type of hemophilia.

IDIOPATHIC THROMBOCYTOPENIC PURPURA (Werlhoff's disease)

Thrombocytopenia is most common form of bleeding disorders due to the quantitative abnormalities of platelets. Because a number of platelets reduce in the blood stream, their function is impaired.

Etiology

Causes of decreased platelet production:

- selective megakaryocytic depression in bone marrow: drug-induced, chemicals.

Infiltration of bone marrow:

• aplastic anemia;

• leukemia;

• myelosclerosis;

- multiple myeloma;

• megaloblastic anemia;

• carcinoma.

Increased destruction of platelets:

- disseminated intravascular coagulation;

• idiopathic thrombocytopenic purpura;

• viral infections - Epstein-Barr virus, HIV;

• bacterial infections - septicemia.

Pathogenesis

Decreased platelet production result from three mechanisms: failure of megakaryocyte maturation, excessive platelet consumption or their sequestration in an enlarged spleen. The pathogenesis of idiopathic thrombocytopenic purpura is associated with activation of the immune system, production of the auto-antibodies, often directed against platelet membrane glycoprotein IIb-IIIa. Platelet destruction results from increased phagocytosis of antigen-antibodies immune complexes adhere to platelet by monocyte-macrophage system of the spleen and liver. The antibody covered platelets premature removal from the circulation. The reason for production of the antibody is unknown, thus this form of disease is defined as idiopathic.

Clinical feature

Idiopathic thrombocytopenic purpura more commonly affects females at an early age. The main complaints are easy bruising in skin and bleeding from mucosa with sudden onset after easy trauma and sometimes spontaneously. Very often symptoms are the bleeding from nose, gastrointestinal tract. lung and kidney hemorrhage, in women - menorrhagia. The course of disease is chronic, with remissions and relapses.

Objective examination. General patient's condition is satisfactory. If bleeding persists for more than some days resulted acute posthemorrhage anemia the patient's condition become grave and required immediately treatment. The main clinical signs are the presence features of skin bruising different size: petechiae, purpura and even hematoma, which located at the anterior part of trunk and extremities. According to the term of bruising appearance may be change of color with different tint: read, blue, green and yellow.

Skin bruising sometimes accompanied with profuse mucosa bleeding and become insidious character because occur posthemorrhage anemia. Spontaneous bleeding docs not usually occur until the platelet count falls below about 30x10⁹/l.

Severe thrombocytopenia results in eye-ground hemorrhage, but intracranial hemorrhage is rare.

Splenomegaly is observered in about 10 % of the cases.