Bleeding disorders

Physiology of hemostasis & coagulation

Vascular Spasm

In vascular spasm, the smooth muscle in the walls of the vessel contracts dramatically.

Formation of the Platelet Plug

platelets clump together, become spiked and sticky, and bind to the exposed collagen and endothelial lining.

Coagulation

Those more sophisticated and more durable repairs are collectively called coagulation, the formation of a blood clot.

Common bleeding and coagulation disorders

hemophilia A (factor VIII deficiency)

hemophilia B (factor IX deficiency)

von Willebrand disease

Heparin induced thrombocytopenia

Platelets role in the coagulation process

Essential mediators that trigger the mechanical pathway of the coagulation cascade upon encountering any damage to the blood vessels.

Define thrombocytopenia & list its causes

Defintion

Thrombocytopenia occurs when your bone marrow doesn’t make enough platelets, which are blood cells that form blood clots to help stop bleeding.

Causes

Bone marrow doesn’t make enough platelets.

Conditions that use up the platelet supply or destroy the platelets

Spleen traps platelets so they can’t circulate through the bloodstream.

Idiopathic Thrombocytopenic Purpura

two clinical subtypes.

acute

Chronic

Etiology

Patients with ITP develop autoantibodies against platelet membrane proteins, specifically glycoprotein (GP) IIb/IIIa complex, GP Ib/IIa, and GP VI.

Clinical manifestation and diagnostic investigations of ITP

Clinical manifestations

Easy bruising

abnormal bleeding

cuts that keep bleeding

Diagnostic investigations

CBC

Blood smear

Bone marrow tests

Difference between childhood and adult ITP

Childhood ITP is often benign and self-limited, whereas ITP in adults tends to be more chronic and difficult to treat.

Describe the management of ITP including recent developments in this field

Management

Corticosteroids

Intravenous immunoglobulin (IVIG)

Platelet transfusion

Recent developments

Sutimlimab

fostamatini

Rozanolixizumab

Side effects and complication of ITP treatment

Steroids

Irritability

stomach irritation

weight gain

Splenectomy

Increased risk of infection

Blood transfusion

hemolytic transfusion reactions

Splenectomy: Immune thrombocytopenic purpura

The spleen is responsible for removing these damaged platelets and therefore removal of the spleen can help to keep more platelets circulating in the body.

Decreases antibody-dependent phagocytosis of platelets

Decreases the half-life of IgG

decreases complement-dependent cytotoxicity