Bloody Battle- Bleeding Disorders
The body engages three processes to stop bleeding: vasoconstriction, gap-plugging by platelets and the coagulation cascade. A fault in any of these process leads to a a bleeding disorder.
When it comes to differentiating bleeding disorders, the pattern of bleeding can yield some clues. Vascular and platelet disorders lead to prolonged bleeding from cuts, bleeding into skin (causing easy bruising, and purpura) bleeding from mucosa membrane (eg epistaxis, bleeding from gums, menorrhagia). Coagulation disorders, on the other hand, cause delayed bleeding into joints and muscles.
Congenital- Osler-weber-rendu syndrome and connective tissue disease such as Elhers- Danlos.
Acquired- Senile purpura, infection ( such as menigococcal, measles, dengue fever), steroids, scruvy (causing perifollicular hemorrhages), Henoch-Schonlein purpura and painful bruising syndrome (where women develop tingling under the skin followed by bruising over the limb/trunk, and resolving without treatment)
Due to decreased marrow production- Aplastic anaemia, megloblastic anemia, marrow infiltration (eg luekemia, myeloma) and marrow suppression (due to cytotoxic drugs and radiotherapy)
Due to excess destruction- Can be either immune or non immune
- Immune includes Immune thrombocytopenia purpura (ITP), and other autoimmune causes such as SLE, drugs such as heparin and viruses.
- Non-immune includes DIC, thrombotic thrombocytopeniaic purpura(TTP) or Hemolytic uremic syndrome
ITP- is cause by antiplatelet autoimmune bodies leading to phagocytic destruction. It is usually acute (in children it presents 2 weeks after infection and causes sudden self limiting purpura) or chronic where it takes an episodic nature of bleeding, purpura, menorrhagia, and epistaxis. It most commonly affects adult women. There is no splenomegaly with ITP. Laboratory investigations show increased megokarytocytes in marrow, antiplatelet autoantibodies may also be present but this is not always the case. No treatment is required for mild cases, however if symptomatic or platelets drop below 20, give prednisolone with the aim to keep platelets above 30. In relapsing cases, splenectomy has an 80% cure rate. If this fails, initiate immunosuppression therapy with azothioprine, etc. Platelet therapy should only be used during life-threatening bleeds or splenectomy as they quickly get destroyed by circulating autoantibodies. Immunoglobulin may temporarily raise the platelet count and can be used for surgery or pregnancy.
Due to decreased platelet function- Myeloproliferative disease, NSAIDs, and increased urea.
Congenital- Haemophilia, von Willebrand’s disease
Acquired- Anticoagulants, liver disease, DIC, vitamin K deficiency.
Haemophilia A- Is a factor VIII deficiency inherited in a recessive X-linked pattern. It usually presents early in life or after surgery/trauma as bleeding into the joints leading to crippling arthropathy and into muscles causing hematomas which can lead to nerve palsies and compartment syndrome due to pressure. Diagnose by increased APTT and decreased factor VIII assay. Those with heamophilia A should avoid NSAIDs and IM injections. In a minor bleed- apply pressure and elevate the part. Desmopressin IV infusion for 20mins can raise factor VIII levels and may be sufficient. Major bleeds (such as heamarthrosis) requires factor VIII levels to be 50% of normal and life threatening bleeds (eg obstructing airways) need factor VIII levels of 100%. Increase factor VIII levels with recombinant factor VIII. Also, insure that the patient receive genetic counselling for future family plans.
Haemophilia B (Christmas disease): Factor IX deficiency which is inherited and behaves clinically like heamophilia A.
Liver disease- produces a complicated bleeding disorder with decreased synthesis of clotting factor, decreased absorption of vitamin K and abnormalities of platelet function.
Malabsorption- Leads to decreased uptake of vitamin K (which is needed for the synthesis of factor II, VII, IX and X) Treat with vitamin K or fresh frozen plasma in the case of acute haemorrhage.