Digestive Diseases and Sciences

, Volume 52, Issue 6, pp 1589–1591 | Cite as

Thrombotic Thrombocytopenic Purpura and Bone Marrow Necrosis Associated with Disseminated Gastric Cancer

  • Zaher K. Otrock
  • Ali T. Taher
  • Jawad A. Makarem
  • Mireille M. Kattar
  • Ghazi Nsouli
  • Ali I. Shamseddine
Case Report


Thrombotic thrombocytopenic purpura (TTP) is a disseminated form of thrombotic microangiopathy. Severe deficiency of the von Willebrand factor (VWF)-cleaving protease, now denoted ADAMTS-13, is known to be responsible for the formation of platelet thrombi in the microvasculature which is believed to be the pathophysiological hallmark of acute idiopathic TTP [1]. The classic pentad of clinical findings consists of fever, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fluctuating neurologic impairment, and renal dysfunction [2]. Although most cases are idiopathic, the association of TTP with malignancy is well recognized [3]. About half of malignancy-associated TTP pertains to gastric carcinoma [4]. It usually occurs at the terminal stage of cancer and is extremely rare as the initial presentation of the disease. Bone marrow necrosis (BMN) is another relatively uncommon entity, partly because it is underdiagnosed. It is defined pathologically as the necrosis of...


Thrombotic thrombocytopenic purpura Bone marrow necrosis Gastric cancer Prognosis Case report 


  1. 1.
    Kremer Hovinga JA, Studt JD, Lammle B (2003) The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP). Pathophysiol Haemost Thromb 33:417–421PubMedCrossRefGoogle Scholar
  2. 2.
    George JN (2000) How I treat patients with thrombotic thrombocytopenic purpura—hemolytic uremic syndrome. Blood 96:1223–1229PubMedGoogle Scholar
  3. 3.
    Rock GA (2000) Management of thrombotic thrombocytopenic purpura. Br J Haematol 109:496–507PubMedCrossRefGoogle Scholar
  4. 4.
    Murgo AJ (1987) Thrombotic microangiopathy in the cancer patient including those induced by chemotherapeutic agents. Semin Hematol 24:161–177PubMedGoogle Scholar
  5. 5.
    Janssens AM, Offner FC, Van Hove WZ (2000) Bone marrow necrosis. Cancer 88:1769–1780PubMedCrossRefGoogle Scholar
  6. 6.
    Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 325:393–397PubMedCrossRefGoogle Scholar
  7. 7.
    Nordstrom B, Strang P (1993) Microangiopathic hemolytic anemia (MAHA) in cancer. A case report and review. Anticancer Res 13:1845–1849PubMedGoogle Scholar
  8. 8.
    Paydas S, Ergin M, Baslamisli F, Yavuz S, Zorludemir S, Sahin B, Bolat FA (2002) Bone marrow necrosis: clinicopathologic analysis of 20 cases and review of the literature. Am J Hematol 70:300–305PubMedCrossRefGoogle Scholar
  9. 9.
    Pirrotta MT, Bucalossi A, Forconi F, Bocchia M, Mazzotta S, Sammassimo S, Gozzetti A, Lauria F (2005) Thrombotic thrombocytopenic purpura secondary to an occult adenocarcinoma. Oncologist 10:299–300PubMedCrossRefGoogle Scholar
  10. 10.
    Gonzalez N, Rios E, Martin-Noya A, Rodriguez JM (2002) Thrombotic thrombocytopenic purpura and bone marrow necrosis as a complication of gastric neoplasm. Haematologica 87:ECR01PubMedGoogle Scholar
  11. 11.
    Antman KH, Skarin AT, Mayer RJ, Hargreaves HK, Canellos GP (1979) Microangiopathic hemolytic anemia and cancer: a review. Medicine 58:377–384PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, Inc. 2007

Authors and Affiliations

  • Zaher K. Otrock
    • 1
  • Ali T. Taher
    • 1
  • Jawad A. Makarem
    • 1
  • Mireille M. Kattar
    • 2
  • Ghazi Nsouli
    • 3
  • Ali I. Shamseddine
    • 1
    • 4
  1. 1.Department of Internal MedicineAmerican University of Beirut Medical CenterBeirutLebanon
  2. 2.Department of Pathology and Laboratory MedicineAmerican University of Beirut Medical CenterBeirutLebanon
  3. 3.Department of Internal MedicineFouad Khoury HospitalBeirutLebanon
  4. 4.Division of Hematology/Oncology, Department of Internal MedicineAmerican University of Beirut Medical CenterBeirut 1107 2802Lebanon

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