IDIOPATHIC THROMBOCYTOPENIC PURPURA
Etiology: Autoimmune, IgG ab against platelets
In kids occus after viral illness and is usually selflimited. It is believed that the body, when making antibodies against a virus, "accidentally" also made an antibody that can stick to the platelet cells. The body recognizes any cells with antibodies as foreign cells and destroys them.
Symptoms: Platelets <100 000, acute bleeding, petequias. If platelets very low, or symptoms give corticosteroids, 2nd line IV immunoglobulin
Etiology: Autoimmune, IgG ab against platelets
In kids occus after viral illness and is usually selflimited. It is believed that the body, when making antibodies against a virus, "accidentally" also made an antibody that can stick to the platelet cells. The body recognizes any cells with antibodies as foreign cells and destroys them.
Symptoms: Platelets <100 000, acute bleeding, petequias. If platelets very low, or symptoms give corticosteroids, 2nd line IV immunoglobulin
THROMBOTIC THROMBOCYTOPENIC PURPURA
Increased platelet consumption
Forms: acquired and congenital.
Classic Pentad: FEVER + ANEMIA + THROMBOCYTOPENIA + RENAL FAILURE + NEURO CHANGE
Associated with metastatic disease, mitomycin and ticlopidine
ADAMTS13 gene defect
Treatment: Plasmapheresis (if not available FFP+corticosteroid)
Increased platelet consumption
Forms: acquired and congenital.
Classic Pentad: FEVER + ANEMIA + THROMBOCYTOPENIA + RENAL FAILURE + NEURO CHANGE
Associated with metastatic disease, mitomycin and ticlopidine
ADAMTS13 gene defect
Treatment: Plasmapheresis (if not available FFP+corticosteroid)
HEMOLYTIC UREMIC SYNDROME
Microangiopathic hemolytic anemia, renal vascular damage. rarely neurological involvement.
Prodrome of bloody diarrhea (EHEC E.coli O157:H7)
Treated as TTP
Microangiopathic hemolytic anemia, renal vascular damage. rarely neurological involvement.
Prodrome of bloody diarrhea (EHEC E.coli O157:H7)
Treated as TTP
DIC
Prolonged: PT/PTT/FDP
Positive D Dimmer, Protamine
: platelet count, fibrinogen
Tx: treat underlying cause
Prolonged: PT/PTT/FDP
Positive D Dimmer, Protamine
: platelet count, fibrinogen
Tx: treat underlying cause
HELLP SYNDROME
Hemolysis, elevated liver enzymes, low platelets
Treatment : Delivery
Hemolysis, elevated liver enzymes, low platelets
Treatment : Delivery
HEPARIN INDUCED THROMBOCYTOPENIA
Ab recognizes heparin/platelet factor IVcomplexes with resultant in activation of platelets.
Onset of thrombocytopenia within 5-10 days of heparin initiation.
Mayor complication: Thrombosis, no bleeding
Treatment: STOP HEPARIN, start direct thrombin inhibitors (argatroban, lepirudin)
Do not give lepirudin in renal insufficiency
Do not give argatroban on liver disease
Ab recognizes heparin/platelet factor IVcomplexes with resultant in activation of platelets.
Onset of thrombocytopenia within 5-10 days of heparin initiation.
Mayor complication: Thrombosis, no bleeding
Treatment: STOP HEPARIN, start direct thrombin inhibitors (argatroban, lepirudin)
Do not give lepirudin in renal insufficiency
Do not give argatroban on liver disease
EVANS SYNDROME
Presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA) in conjunction with immune-mediated thrombocytopenia (ITP), with no known underlying etiology.
TX: Prednisone or immunoglobulin
Presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA) in conjunction with immune-mediated thrombocytopenia (ITP), with no known underlying etiology.
TX: Prednisone or immunoglobulin
MYELODYSPLASIA
* Clonal stem cell disorder in elderly
* Prognosis depends on number of blasts
* Look in the perispheral smear for: Dimorphic RBC population, macrocytic RBC, Pseudo Pelger Huet anomaly, large agranular platelets, decreased reticulocyte count, ringed sideroblasts.
*Can turn to AML
* 5q- syndrome: usually in females, thrombocytosis. Favorable prognosis
* Clonal stem cell disorder in elderly
* Prognosis depends on number of blasts
* Look in the perispheral smear for: Dimorphic RBC population, macrocytic RBC, Pseudo Pelger Huet anomaly, large agranular platelets, decreased reticulocyte count, ringed sideroblasts.
*Can turn to AML
* 5q- syndrome: usually in females, thrombocytosis. Favorable prognosis
BLOOD TRANSFUSION REACTIONS
Leading cause of transfusion reaction: fever!!!!! (not hemolysis)
* ABO Incompatibility: early reaction (clerical error, wrote A instead of B). Within an hour of blood transfusion pt become dyspneic, low back pain, fever, dark urine. Tx hydrate and diuresis, vasopressors.
* Rh incompatibility: delayed
* Fever + hypotension: Bacterial contamination on blood
*Transplant patient: give CMV negative and irradiated blood
* If hypotension after PRBC transfusion: IgA deficiency
* If itchy after blood transfusion: next blood transfusion give washed PRBC
* TRALI ( Transfusion Related Acute Lung Injury) : leading cause of death. Needs intubation.
Symptoms develop within 6 hours of a transfusion, dyspnea and tachypnea. There may be associated fever, cyanosis, and hypotension. . CXR : pulmonary edema unassociated with heart failure, with bilateral patchy infiltrates.
Leading cause of transfusion reaction: fever!!!!! (not hemolysis)
* ABO Incompatibility: early reaction (clerical error, wrote A instead of B). Within an hour of blood transfusion pt become dyspneic, low back pain, fever, dark urine. Tx hydrate and diuresis, vasopressors.
* Rh incompatibility: delayed
* Fever + hypotension: Bacterial contamination on blood
*Transplant patient: give CMV negative and irradiated blood
* If hypotension after PRBC transfusion: IgA deficiency
* If itchy after blood transfusion: next blood transfusion give washed PRBC
* TRALI ( Transfusion Related Acute Lung Injury) : leading cause of death. Needs intubation.
Symptoms develop within 6 hours of a transfusion, dyspnea and tachypnea. There may be associated fever, cyanosis, and hypotension. . CXR : pulmonary edema unassociated with heart failure, with bilateral patchy infiltrates.
POLYCYTHEMIA VERA
Elevated hematocrit level (greater than 60% in men and 56% in women. )
Decreased serum erythropoietin level
Normal arterial oxygen saturation
JAK2 mutation.
TX: Therapeutic phlebotomy ( goal Hct less than 45% for men and less than 42% for women) Plus low dose aspirin.
If no response to phlebotomy or cannot tolerate it, hydroxyurea.
Elevated hematocrit level (greater than 60% in men and 56% in women. )
Decreased serum erythropoietin level
Normal arterial oxygen saturation
JAK2 mutation.
TX: Therapeutic phlebotomy ( goal Hct less than 45% for men and less than 42% for women) Plus low dose aspirin.
If no response to phlebotomy or cannot tolerate it, hydroxyurea.
SICKLE CELL DISEASE
* Stroke and sickle cell: Strokes recur in up to 70% of patients with sickle cell disease. Tx: monthly erythrocyte transfusions (hypertransfusions) of two units decreases stroke recurrence by 50% in patients with sickle cell disease.
* Stroke and sickle cell: Strokes recur in up to 70% of patients with sickle cell disease. Tx: monthly erythrocyte transfusions (hypertransfusions) of two units decreases stroke recurrence by 50% in patients with sickle cell disease.