Disorders of Excess Bleeding

Summary of bleeding disorders in children:

7. Idiopathic thrombocytopenic purpura (ITP) (illustration)
1. Etiology: unknown, but often occurs one to three weeks after a febrile, viral illness
2. Pathophysiology
1. auto-immune disorder
2. platelets are killed and fewer are made
3. may be acute and self-limiting; or chronic
3. Findings: excessive bruising, petechiae, internal bleeding
4. Therapeutic management: steroids, immunoglobulins (IVIG), anti-D antibody, splenectomy for chronic disease
   
8. Von Willebrand's disease
1. Etiology: congenital (autosomal dominant) bleeding disorder; affects both males and females
2. Pathophysiology
1. body makes too little von Willebrand's factor and factor VIII
2. prolonged bleeding time
3. Findings: excessive bleeding of mucous membranes: gums, epistaxis (nosebleed), menorrhagia
4. Therapeutic management: replacement of missing clotting factor, and/or administration of antidiuretic: desmopressin acetate (DDAVP)


9. Aplastic anemia (illustration)
1. Etiologies
1. congenital (example: Faconi's anemia)
2. acquired due to exposure to overwhelming infection (hepatitis, HPV)
3. causative agents such as antineoplasticagents, chemicals, chloramphenicol
2. Pathophysiology
1. bone marrow stops making erythrocytes, leukocytes, and platelets.
2. result: pancytopenia
3. Findings: petechiae, bruising, pallor, fatigue, myelosuppression
4. Therapeutic management
1. antithymocyte globulin (ATG), antilymphocyte globulin (ALG)
2. if severe, bone marrow transplant is treatment of choice
   
10. Hemophilia (illustration )
1. Etiology: x-linked recessive disorder
2. Pathophysiology: missing or defective factor VIII or factor IX blood components necessary for blood coagulation
3. Findings:
1. mild-severe prolonged bleeding; most often in muscles and joints (hemathrosis)
2. longterm loss of range of motion of affected joints
4. Therapeutic management
1. replacement of missing clotting factor, factor VIII concentrate
2. desmopressin acetate, a diuretic (DDAVP)
3. prophylactic treatment with clotting factor before surgery and some other procedures
4. ice, rest, elevation, immobilization, pressure to bleeding site
   
11. Disseminated intravascular coagulation (DIC) (illustration )
1. Etiology
1. secondary disorder of coagulation that complicates other disorders
2. triggered by endothelial damage such as trauma, shock, infections, hypoxia, liver disease
2. Pathophysiology
1. the first stage of the coagulation process is abnormally stimulated
2. clotting mechanism is triggered in circulation, thrombin is generated in greater amounts than the body can neutralize
3. rapid conversion of fibrinogen to fibrin with aggregation and destruction of platelets
4. local and widespread fibrin deposition in blood vessels causes obstruction and necrosis
5. fibrinolytic mechanism causes extensive destruction of clotting factors
3. Findings
1. bleeding, bruising, petechiae
2. altered serum levels of clotting-related factors (increased PT, PTT, TT, decreased platelets, degraded fibrinogen)
3. clotting, hypoxemia, intracranial hemorrhage, progressive organ failure
4. Therapeutic management
1. control of underlying etiology
2. factor replacement
3. platelets, fresh frozen plasma (FFP) and RBC transfusions
4. vitamin K
5. oxygen
   
1. Pediatric Oncology

1. Cardinal findings of cancer in children
1. Unusual mass
2. Pallor
3. Sudden tendency to bruise
4. Rapid, unexplained weight loss
5. Change in vision or eye
6. Recurrent fever
7. Persistent headache, often with vomiting
8. Change in balance or gait
   
2. Leukemias - most common cancer in children (illustration 1   illustration 2 )
1. Definition: cancer of blood-forming tissues
2. Pathophysiology of acute lymphoid leukemia (ALL) and acute myelogenous leukemia (AML)
1. malignancies of the bone marrow and lymphatic system
2. unrestricted proliferation of immature WBCs
3. leukemia cells demonstrate the neoplastic properties of solid cancers
4. symptoms caused by infiltration and replacement of any tissue of the body with non-functional leukemia cells.
5. highly vascular organs such as spleen and liver are most severely affected
3. Findings
1. acute or insidious onset,
2. anemia, thrombocytopenia, infection, bleeding
3. lymphadenopathy, hepatosplenomegaly, bone or joint pain
4. Therapeutic management
1. combination chemotherapy
2. radiation if CNS involved
3. bone marrow transplant