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THROMBOCYTOPENIC PURPURA ICD-10: D69.3

  • Thrombocytopenic purpura (TP) is characterized by cutaneous hemorrhages occurring in association with a reduced platelet count.

  • Occur at sites of minor trauma/pressure (platelet count <40,000/μL) or spontaneously (platelet count <10,000/μL).

  • Due to decreased platelet production, splenic sequestration, or increased platelet destruction.

  • Decreased platelet production. Direct injury to bone marrow, drugs (cytosine arabinoside, daunorubicin, cyclophosphamide, busulfan, methotrexate, 6-mercaptopurine, vinca alkaloids, thiazide diuretics, ethanol, estrogens), replacement of bone marrow, aplastic anemia, vitamin deficiencies, and Wiskott–Aldrich syndrome.

  • Splenic sequestration. Splenomegaly and hypothermia.

  • Increased platelet destruction. Immunologic: Autoimmune TP, drug hypersensitivity (sulfonamides, quinine, quinidine, carbamazepine, digitoxin, or methyldopa), after transfusion. Nonimmunologic: Infection, prosthetic heart valves, disseminated intravascular coagulation, and thrombotic TP (microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal disease).

  • Skin Lesions. Petechiae: Small (pinpoint to pinhead), red, nonblanching macules that are not palpable and turn brown as they get older (Fig. 20-1); later acquiring a yellowish-green tinge. Ecchymoses: Black-and-blue spots; larger area of hemorrhage. Vibices: Linear hemorrhages (Fig. 20-1), due to trauma or pressure. Most common on legs and upper trunk, but may occur anywhere.

  • Mucous Membranes. Petechiae: Most often on palate (Fig. 20-2) and gingival bleeding.

  • General Examination. Possible CNS and internal hemorrhage, anemia.

  • Laboratory Hematology. Thrombocytopenia.

  • Serology. Rule out HIV disease; antibodies against ADAMTS 13 (a protease).

  • Lesional Skin Biopsy (bleeding usually can be controlled by suturing biopsied site) to rule out vasculitis.

  • Differential diagnosis. Senile purpura, purpura of scurvy, progressive pigmentary purpura (Schamberg disease), purpura following severe Valsalva maneuver (coughing, vomiting/retching), traumatic purpura, factitial or iatrogenic purpura, and vasculitis.

  • Management. Identify underlying cause and correct, if possible. Oral glucocorticoids, high-dose IV immunoglobulins, or platelet transfusion for chronic (Immune Thrombocytopenic Purpura) ITP (splenectomy may be indicated).

FIGURE 20-1

Thrombocytopenic purpura Multiple petechiae on the upper arm of an HIV-infected 25-year-old male were the presenting manifestation of his disease. The linear arrangement of petechiae at the site of minor trauma is called vibices.

FIGURE 20-2

Thrombocytopenic purpura Can first manifest on the oral mucosa or conjunctiva. Here, multiple petechial hemorrhages are seen on the palate.

DISSEMINATED INTRAVASCULAR COAGULATION ICD-10: D65

  • Disseminated intravascular coagulation (DIC) is a widespread blood clotting disorder occurring within blood vessels.

  • Associated with a wide range of clinical circumstances: Bacterial sepsis, obstetric complications, disseminated malignancy, and massive trauma.

  • Manifested by purpura fulminans (cutaneous infarctions and/or acral gangrene) or bleeding from multiple sites.

  • The spectrum of clinical symptoms associated with DIC ranges from relatively mild and subclinical to explosive and life threatening.

  • Synonyms: Purpura fulminans, consumption coagulopathy, defibrination syndrome, and coagulation fibrinolytic syndrome.

EPIDEMIOLOGY

AGE OF ONSET All ages; occurs in children.

ETIOLOGY AND PATHOGENESIS

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