1. Protein C (thrombin --> thrombomodulin, which activates C, which along with Protein S, inactivates Va and VIIIa)
2. Antithrombin III (neutralizes activated enzymes with heparan sulfate, target of heparin)
3. Fibrinolytic system (tPa activates plasminogen into its plasmin form)

GpIIb-IIIa.

When platelets are activated, they release ADP, which binds to the glycoprotein. This causes conformational change and, subsequently, activation. It is deficient in Glanzmann disease.

Excessive, dysregulated activation of coagulation system. Factors and platelets are consumed. Thrombosis in mild cases, hemorrhage in severe.

Common causes are sepsis from G- bacteria, and obstretical complications (necrotic tissue from placenta consumes clotting factor)

Treat underlying condition

Identify the most likely causes:

1. Patient is factor VII and X deficient, with normal V and VIII
2. Patient is factor V, VII, X deficient, with normal VIII
3. Patient is factor V, VII, VIII, X deficient

1. Vitamin K deficiency
2. Liver disease (factors all made in liver, but VIII is preserved in cirrhosis)
3. DIC

Performs carboxylation that allow calcium binding.

Poor diet (lacking in leafy green vegatables), broad-spectrum antibiotics (bacteria responsible for Vit. K absorption)

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1. Antithrombin III deficiency
2. Protein C or S deficiency
3. Factor V:Leidin (common mutation that prevents Protein C mediated degradation of factor V)
4. Prothrombin gene mutation (prothrombin 20210- excessive half-life of prothrombin mRNA)

Initial period (5 days) - Heparin to reduce new fibrin deposition and allow natural lysis.

Chronic (6-12 months) - Warfarin to prevent recurrence

Normally, 1/3 platelets sequestered.

Splenomegaly can lead to 90% sequestration, although typically does not results in a plt count below 50k. Cirrhosis, and portal htn can lead to thrombocytopenia.

1. ITP (immune mediated platelet destruction)
2. Drug-induced thrombocytopenia

1. TTP (thrombotic cytopenia purpura, due to ADAMTS13/vWF cleaving protein deficiency)
2. HIT (heparin induced thrombosis, antibodies directed against PFIV-Heparin fusion protein leads to platelet activation)
3. DIC (uncontrolled prudction of thrombin and fibin deposition)

TTP

Fragmented/cleaved cells (schistocytes)

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High LDH and retic count.

Either congenital deficiency in ADAMST13 or autoimmune response directed against it. The protein is produced on endothelial cells, and cleave vWF. Without cleavage, large multimers cause spontaneous thrombi.

Plasmapherisis removes these large multimers and potentially removes antibodies directed against ADAMST13. It can also supply missing vWF protease.

Iron deficiency generally more hypochromic, microcytic.

Definitive assay is transferrin receptor assay (increased Tf in iron deficiency)

Also, ACD typically responds well to EPO

1. Vascular obstruction (stroke, retinal disease, necrosis of bones)
2. Renal damage (papillary necrosis)
3. Priapism (painful, prolonged erection)
4. Osteomyletis from salmonella infection
5. Ankle ulcers

1. Plasmodium induce sickling in infected erythrocytes, leading to targeted removal by spleen.
2. Limited oxygen availability in hemoglobin-deprived erythrocytes prevent plasmodium proliferation.

1. Presents as hemolytic anemia (hematocrit ~30%).
2. Target cells and spherocytes present (also present in carriers).
3. Crystals are observed in RBCs.
4. Generally well.

1. Slightly highe rhematocrit (28-33%)
2. Splenomegaly
3. Presence of target cells and spherocytes in addition to sickle cells.

1. Microcytic, hypochromic peripheral RBCs if just carrier.
2. In untreated major intramedullary hemolysis, ineffective erythropoiesis and extramedullary hematopoeisis (bone abnormalities)