Page 2167 - Hematology_ Basic Principles and Practice ( PDFDrive )

1920 Part XII Hemostasis and Thrombosis

TABLE Differential Diagnosis of Acquired Bleeding Problems
128.3
Disorder Comments
Drug induced Aspirin, NSAIDs, other platelet function inhibitors (e.g., P2Y 12 and α IIb β 3 inhibitors), anticoagulants, fibrinolytic drugs, and
antidepressants are common causes
Acquired factor The causes can be immune (e.g., acquired factor VIII deficiency, acquired factor V deficiency) or nonimmune. Reductions in
deficiencies multiple factors can result from vitamin K deficiency, treatment with vitamin K antagonists, liver disease, hemodilution, and
rarely snakebites. Severe acquired hypofibrinogenemia is commonly caused by a postpartum coagulopathy or severe liver
disease. Prothrombin deficiency occurs with some lupus anticoagulants. Amyloidosis can cause an acquired factor X
deficiency, which may be associated with reductions in other coagulation factors synthesized in the liver if the liver is
involved.
Disseminated The manifestations can include thrombocytopenia, consumption of coagulation factors, including fibrinogen, and impairment
intravascular of hemostatic mechanisms from the fibrin/fibrinogen degradation products. Causes are wide ranging and include postpartum
coagulation consumptive states, prostate and other cancers, and snakebites.
Acquired von The cause can be immune (often in association with an IgG paraprotein) or nonimmune (e.g., increased proteolysis of von
Willebrand disease Willebrand factor with stenotic aortic valvular disease).
Immune Bleeding is usually influenced by the extent of the thrombocytopenia. Some autoantibodies interfere with platelet membrane
thrombocytopenia receptor function, causing bleeding disproportionate to the thrombocytopenia.
Non–drug induced, The cause can be immune (see earlier) or nonimmune, typically from bone marrow disorders, although secretion defects can
acquired platelet be secondary to Cushing syndrome or hypothyroidism.
function disorders
Liver disease Liver disease can cause thrombocytopenia, deficiencies of coagulation factors, hypofibrinogenemia and dysfibrinogenemia, and
increased fibrinolysis. In mild liver disease, factor VII and sometimes factors XI and XII are low. Fibrinogen is often
increased in early liver disease, and if low, the finding suggests severe liver disease.
Renal disease Anemia is an important predictor of uremic bleeding. Uremic bleeding is typically associated with severe renal impairment.
Hypothyroidism Hypothyroidism can cause an acquired von Willebrand disease and acquired defects in platelet function.
Cushing syndrome This syndrome should be suspected when there are symptoms and findings suggestive of Cushing syndrome or treatment with
systemic or topical glucocorticoids.
Surgical bleeding This is often a diagnosis of exclusion, although the procedural notes sometimes document that a technical problem was
encountered that led to abnormal bleeding.
Vitamin K deficiency Newborns are at risk, as are individuals with malabsorption and/or receiving broad-spectrum antibiotics that reduce vitamin K
production by reducing gut bacteria. Older adults are also at greater risk for developing vitamin K deficiency, because of
reduced stores from poorer intake of vitamin K. If the patient does not respond to parenteral vitamin K, other causes should
be considered.
Vitamin C deficiency This diagnosis should be considered when there is lethargy with skin and gum bleeding (perifollicular hemorrhages, gum
(scurvy) bleeding with swelling). The condition is rare in developed countries. The cause is usually a very poor diet or malabsorption.
IgG, Immunoglobulin G; NSAID, nonsteroidal antiinflammatory drug.

Case 6: Illustration of Changes in Bleeding Outcomes With Treatment
A 38-year-old woman was evaluated for a bleeding disorder. Her family with multiple aggregation abnormalities, with no evidence of von Wil-
physician had already excluded the possibility of von Willebrand disease. lebrand disease. She underwent additional surgical procedures, using
The patient had a long-standing history of massive bruises, often without desmopressin treatment to reduce her bleeding risks, with no abnormal
recollection of trauma, prolonged nosebleeds requiring medical attention bleeding. Her menorrhagia was controlled with tranexamic treatment.
since early childhood, prolonged bleeding from minor cuts, and severe She self-administered desmopressin treatment to control nosebleeds,
bleeding requiring blood transfusions with many surgical procedures. with good effect. This case illustrates that treatment affects bleeding
She had a history of recurrent iron-deficiency anemia, menorrhagia outcomes and the importance of evaluating for common defects in
requiring medical therapies, and immediate postpartum bleeding. Her hemostasis.
father had a history of bleeding problems, but the cause of the bleeding For more information on therapies for specific disorders, see the
problem in the family was unknown. The history suggested an inherited chapters on hemophilia (Chapters 135 and 136), rare coagulation factor
disorder, possibly a platelet function disorder or a form of von Willebrand deficiencies (Chapter 137), von Willebrand factor (Chapter 138), and
disease. The testing indicated that she had a platelet secretion defect platelet disorders (Chapters 125 and 130–132).

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