Blood

Disorders

Hemophilia: This can be the result of a number of different factors involved in the clotting mechanism. The most common is a factor VIII deficiency which is an X-linked( involving the X or sex chromosome) recessive trait. The most common S&S include prolonged bleeding which can be classified as mil, moderate or severe depending on the level of factor VIII activity remaining. Subcutaneous fat later and intramuscular bleeding are common but the effects are especially critical if the bleeding occurs in the airways or intracranially or around the spinal cord. GI bleeds can also be severe d/t the potential for very large scale loss of blood. Hemarthrosis or bleeding into the joint - especially in the hips,knees, ankles or elbows can be a very serious problem. It is common because the high level of mobility of these joints commonly leads to blood vessel damage which is not a problem for a normal clotting situation. In hemophilia this bleeding into the joints can lead to fibrosis and bone changes and to severe contractures - al of which can lead to severe pain and loss of joint mobility. LEUKEMIAS Leukemias are a group of malignant neoplasms of one class of white blood cells (WBCs) in the bone marrow. The bone marrow, other WBC types and RBCs are replaced by the unregulated, proliferating, undifferentiated and non-functional neoplastic cells which eventually enter the general circulation and infiltrate the spleen, liver, brain and lymph nodes. Leukemias are classified by the specific cell line that has been transformed (ie. Lymphocytes, moncytes, or granulocytes) and can present either acutely or chronically.

Acute Leukemia Acute Leukemias: Abrupt onset characterized by a high proportion of very immature, non-functional cells and marked signs and complications. More common in children and young adults.

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Early Manifestations: Infection that is unresponsive to treatment, excessive bleeding and bruising, anemia. Later Manifestations: Severe bone pain, weight loss and fatigue, enlarged lymph nodes, spleen and liver. If leukemic cells enter the CNS see headache, visual disturbance, drowsiness or vomiting. Prognosis is good (>75% long-term survival rate) for acute childhood leukemias, mainly due to their responsiveness to chemotherapy such as vincristine and vinblastine derived from the periwinkle plant (Vinca) from Madagascar. The prognosis for adult acute leukemias ( eg.AML) is not so encouraging since the treatments with chemotherapy are much less successful. Bone marrow transplants can be successful if all abnormal cells can be elimintated and it may become even more promising as we get better at screening out abnormal cells in cultured autotransplants.

Chronic Leukemias: Onset is insidious and cancer progresses slowly with mild or fewer signs. Characterized by more highly differentiated/mature neoplastic cells. Chronic leukemais are more common in adults. Early Manifestations: Fatigue, weakness, and frequent infections. Later Manifestations: Severe fatigue, swollen lymph nodes, severe fatigue, persistent infections, pain, pallor, splenomegaly, and weight loss. Risk Factors for Leukemia: 1) Genetic predisposition. 2) Chromosomal abnormalities e.g. Downs Syndrome. 3) Exposure to radiation, chemotherapy, and bone-marrow suppressing drugs.

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Disorders

4) Exposure to environmental and industrial agents e.g. pesticides, benzene (eg. In gasoline). 5) Certain viral infections are suspected.