Hematology and Oncology Book

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Protein C Deficiency

Aka: Protein C Deficiency, Protein S Deficiency
  1. Pathophysiology
    1. Protein C neutralizes factors V and VIII
      1. Deficiency inherited Autosomal Dominant
      2. Venous thrombosis occurs if functional levels <50%
    2. Protein S is Cofactor for Protein C
      1. Venous thrombosis occurs if functional levels <60%
    3. Protein C and Protein S are both Vitamin K dependent
  2. Complications
    1. Warfarin induced skin necrosis
      1. Protein C (short half life)
        1. Microvascular thrombi to skin
      2. Administer Heparin before Warfarin
        1. Eliminates risk of this complication
    2. Purpura fulminans
      1. Infants Homozygous for Protein C Deficiency

Protein S Deficiency (C0242666)

Definition (NCI) A rare thrombophilia disorder characterized by deficiency of protein S. It results in venous thromboembolism.
Definition (MSH) An autosomal dominant disorder showing decreased levels of plasma protein S antigen or activity, associated with venous thrombosis and pulmonary embolism. PROTEIN S is a vitamin K-dependent plasma protein that inhibits blood clotting by serving as a cofactor for activated PROTEIN C (also a vitamin K-dependent protein), and the clinical manifestations of its deficiency are virtually identical to those of protein C deficiency. Treatment with heparin for acute thrombotic processes is usually followed by maintenance administration of coumarin drugs for the prevention of recurrent thrombosis. (From Harrison's Principles of Internal Medicine, 12th ed, p1511; Wintrobe's Clinical Hematology, 9th ed, p1523)
Concepts Disease or Syndrome (T047)
MSH D018455
ICD10 D68.59
SnomedCT 1563006
English Deficiencies, Protein S, Deficiency, Protein S, Protein S Deficiencies, Protein S Deficiency, DEFIC PROTEIN S, PROTEIN S DEFIC, protein S deficiency, protein S deficiency (diagnosis), Protein S Deficiency [Disease/Finding], deficiency protein s, s protein deficiency, protein s deficiencies, protein s deficiency, Hereditary Thrombophilia Due To Protein S Deficiency, Protein S Deficiency Disease, Protein S deficiency, Protein S deficiency disease, Protein S deficiency disease (disorder)
Italian Deficit di proteina S, Carenza di proteina S
Swedish Protein S-brist
Czech protein S - nedostatek, Deficit proteinu S
Finnish Proteiini S:n puutos
Russian BELKA S NEDOSTATOCHNOST', БЕЛКА S НЕДОСТАТОЧНОСТЬ
Japanese プロテインS欠乏症, プロテインSケツボウショウ, プロテインS欠損症, Protein S欠損症, 蛋白質S欠損症
Polish Niedobór białka S
Hungarian Protein S hiány
Norwegian Protein S-mangel
Spanish deficiencia de proteína S (trastorno), deficiencia de proteína S, enfermedad por deficiencia de proteína S (trastorno), enfermedad por deficiencia de proteína S, Deficiencia de proteína S, Deficiencia de Proteína S
Portuguese Deficiência de proteína S, Deficiência de Proteína S
Dutch proteïne S-deficiëntie, Deficiëntie, proteïne-S-, Proteïne-S-deficiëntie
German Protein S-Mangel, Protein-S-Mangel
French Déficit en protéine S
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Protein C Deficiency (C0398625)

Definition (NCI) A rare thrombophilia disorder characterized by deficiency of protein C. It results in venous thromboembolism.
Definition (MSH) An absence or deficiency in PROTEIN C which leads to impaired regulation of blood coagulation. It is associated with an increased risk of severe or premature thrombosis. (Stedman's Med. Dict., 26th ed.)
Concepts Disease or Syndrome (T047)
MSH D020151
ICD10 D68.59
SnomedCT 76407009
Swedish Protein C-brist
English PROTEIN C DEFIC, DEFIC PROTEIN C, protein C deficiency (diagnosis), Deficiencies, Protein C, Deficiency, Protein C, Protein C Deficiencies, Protein C Deficiency, protein C deficiency, Protein C Deficiency [Disease/Finding], c protein deficiency, protein c deficiency, c deficiency protein, deficiency protein c, Hereditary Thrombophilia Due To Protein C Deficiency, Protein C Deficiency Disease, Protein C deficiency, Protein C deficiency disease, Protein C deficiency disease (disorder)
Finnish Proteiini C:n puutos
Russian BELKA C NEDOSTATOCHNOST', БЕЛКА C НЕДОСТАТОЧНОСТЬ
Czech Deficit proteinu C, protein C - nedostatek, nedostatek proteinu C, deficit proteinu C
Japanese プロテインC欠乏症, プロテインCケツボウショウ, プロテインC欠損症, PC欠乏症, プロティンC欠乏症, プロテインC欠乏症
Polish Niedobór białka C
Hungarian Protein C hiány
Croatian Protein C, nedostatak
Norwegian Protein C-mangel
Spanish deficiencia de proteína C (trastorno), deficiencia de proteína C, enfermedad por deficiencia de proteína C (trastorno), enfermedad por deficiencia de proteína C, Deficiencia de Proteína C
Portuguese Deficiência de proteína C, Deficiência de Proteína C
Dutch proteïne C-deficiëntie, Deficiëntie, proteïne-C-, Proteïne-C-deficiëntie
French Carence en protéine C, Déficit en protéine C
German Protein C Mangel, Protein C-Mangel
Italian Deficit di proteina C
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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