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Disseminated intravascular coagulopathy (DIC)

Secondary to some other process: sepsis, trauma, metastatic malignancy, obstetric causes

Can be relatively mild indolent course or severe life-threatening process: ongoing coagulation and fibrinolysis can cause thrombosis or hemorrhage; consumption of coagulation factors is seen as prolonged PT and PTT

Treatment aimed at underlying cause. No proven specific treatment for the coagulation problem: if bleeding, replace factors and fibrinogen with fresh-frozen plas-plasma or cryoprecipitate, if clotting, consider anticoagulate with heparin

Thrombocytopenic thrombotic purpura (TTP)

Multiple causes, many seemingly trivial: drugs/infection lead to endothelial injury and release of von Willebrand factor, triggering formation of microvascular thrombi

May present as septic-appearing patient with fever, altered mental status, thrombocytopenia, microangiopathic hemolytic anemia, and renal failure. Previously a very high mortality, mainly because of CNS involvement. Normal PT and PTT

Plasmapheresis (removal of the excess/abnormal vWF). most patients recover. Corticosteroids

Immune thrombocytopenic purpura (ITP)

Antiplatelet antibody leading to platelet destruction

Children: following a viral illness with resolution: in adults, a more indolent course with progression and rarely spontaneous resolution. Isolated thrombocytopenia, normal PT, PTT. Increased megakaryocytes on bone marrow aspiration

Oral corticosteroids; splenectomy if resistant to steroids: possible role for immunosuppressants. Intravenous immunoglobulin

Comprehension Questions

[58.11 A 28-year-oid woman complains of excessive bleeding from her gums and has petechiae. Her neck reveals bilateral supraclavicular adenopathy. The chest radiograph shows a mediastinal mass but no pulmonary lesions. Which of the following is the most likely etiology?

A. Immune thrombocytopenia purpura

B. Systemic lupus erythematosus

C. Drug-induced thrombocytopenia

D. Lymphoma

|58.2] A 50-year-old man has been treated for rheumatoid arthritis for many years. He currently is taking corticosteroids for the disease. On examination. he has stigmata of rheumatoid arthritis and some fullness on his left upper abdomen. His platelet count is slightly low at 105,000/mm3. His white blood cell count is 3,100/mm3 and hemoglobin level 9.0 g/dL. Which of the following is the most likely etiology of the thrombocytopenia?

A. Steroid induced

B. Splenic sequestration

C. Rheumatoid arthritis autoimmune induced

D. Prior gold therapy

[58.3] A 30-year-old woman with ITP has been taking maximum corticosteroid doses and still has a platelet count of 20,000/mm1 and frequent bleeding episodes. Which of the following should she receive before her splenectomy?

A. Washed leukocyte transfusion

B. Intravenous interferon therapy

C. Pneumococcal vaccine

D. Bone marrow radiotherapy

|58.4| A 65-year-old man who has a prosthetic heart valve is hospitalized and placed on IV heparin for anticoagulation. He drinks one glass of wine each weekend and has been diagnosed with osteoarthritis for which he takes acetaminophen. His platelet count is 32.000/mm\ Which of the following is the most likely cause of the thrombocytopenia?

A. Prosthetic heart valve

B. Alcohol intake

C. Acetaminophen

D. Heparin

Answers

|58.1] D. The mediastinal mass and supraclavicular lymphadenopathy on chest radiograph are suspicious for lymphoma, which may affect the bone marrow, leading to decreased platelet production.

|58.2] B. This patient with rheumatoid arthritis likely has splenomegaly, also known as Felty syndrome. Splenomegaly from any etiology may cause sequestration of platelets, leading to thrombocytopenia.

[58.3] C. Patients who undergo splenectomy are at risk for infections of encapsulated organisms such as Streptococcus pneumoniae and thus should receive the pneumococcal vaccine. It usually is given 2 weeks prior to splenectomy so that the spleen can help in forming a better immune response.

[58.4) D. Heparin is associated with thrombocytopenia. Treatment consists of stopping the heparin.

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