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Acquired hemophilia – A pada penderita Waldenstrom’s Macroglobulinemia: laporan kasus



Abstract

Background: Acquired hemophilia A (AHA) is a rare acquired bleeding disease, characterized by autoantibody against factor VIII. Bleeding usually in the skin and mucosal. AHA frequently in the elderly, in male and female and is associated with several conditions, such as autoimmune, pregnancy, drug reactions, solid tumor and monoclonal gammopathy. Half of patients are idiopathic. AHA is diagnosed in patients without previous personal or family bleeding history in which prolonged aPTT is not corrected after mixing and accompanied by a decrease in FVIII levels. Specific antibody that plays a role in inhibiting FVIII activity can be seen from the immonotyphing examination. Waldenstrom’s Macroglubulinemia (WM) is a one of disorders that can cause AHA.

Case Description: A 56-year-old man came with a weak and pale 10 days of hospitalized. Physical examination shown anemia, gum bleeding, lien palpable in Shuffner-1. Laboratory shown bisitopenia and leukocytosis, prolonged aPTT is not corrected after mixing with decrease FVIII levels. (BMP) support Chronic Lympocytic Leukemia/Lymphoma/ Smoldering Myeloma. SPE M-Spike with Immunotyphing examination of IgM lamda monoclonal. History, physical examination, laboratory, BMP, SPE, and immunotyping shown monoclonal gammophaty which cause Acquired Hemophilia A. Bethesda assay examination recommended if FVIII therapy is not responding. Lymphoplasmasitoid cells infiltration and monoclonal IgM production establishes diagnosis of WM.

Conclusion: Spontaneous hemorrhage without prior history with prolonged aPTT is not corrected after mixing and followed by a decrease in FVIII leves can lead to (by the presence inhibitor factors) coagulation disorders.
Keywords: Acquired Hemophilia A monoclonal gammopathy Waldenstrom macroglobulinem

References

  1. Bakta, IM. Hemophilia A yang didapat: Suatu Tinjauan tentang Diagnosis dan Manajemen. Warmadewa Media Jurnal. 2017;2(2): 44-51.
  2. Mariasanta N, Simao R, Maria Rosa L, Melenia C, Salvatrice M, Maria G, et al. Acquired Hemophilia A as Early Manifestation of Multiple Myeloma. J Hematol Mult Myeloma. 2017;2(1): 1006.
  3. Knoeble P, Marco P, Baudo F, Collins P, Huth-Kuhne A, Nemes L, et al. Demographic and clinical data in acquired hemophilia A: results from the European Hemophilia Registry (EACH2). J Thromb Haemost 2012;10(4): 622.
  4. Nicholas B. Abt, Michael B. Streiff, Christian B. Gocke, Thomas S. Kickler, Sophie M. Lanzkron. Idiopathic Acquired Hemophilia A with undetectable Factor VIII Inhibitor. Hindawi Journal 2014, article ID 484563.
  5. Janbain M, Leissinger CA, Krusse-Jarres R. Acquired hemophilia A: emerging treatment options. J Blood Med 2015;6: 143.
  6. Sakurai Y, Takeda T. Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorders. J Immunol Res 2014, Article ID 320674.
  7. Collins P, Baudo F, Knoeble P, Levesque H, Nemes L, Pellegrini F, et al. Immunosupression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood 2012;120(1): 47-55.
  8. Baudo F, Collins P, Huh-Kuhne A, Levesque H, Marco P, Nemes L, et al. Management of bleeding in acquired hemophiliaA: results from the European Acquired Haemophilia(EACH2) Registry. Blood 2012;120(1): 39-46.
  9. Yang Y, Xue F, Shi H, Wang H, Zhang L, Ji M, et al. Acquired Hemophilia A: Retrospective Analysis of 49 Cases From a Single Chinese Hemophilia Center. Clin Appl Thromb/Hemost 2015;21(1): 35-40.
  10. Franchini M. Rituximab in the treatment of adult acquired hemophilia A: A systematic review. Criticals Rev in Oncol/Hematol 2007;63: 47-52.
  11. Collins P, Baudo F, Huth-Kuhne A, Ingerslev J, Kessler CM, Castellano EM, et al. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Reseach Notes 2010;3: 161.
  12. Endah P, Riadi W. Penyakit Waldenstrom Makroglobulinemia. Jurnal Kedokteran Yarsi. 2015;23(1): 056-066.

How to Cite

Alamsyah, A., & Wulanjani, H. A. (2019). Acquired hemophilia – A pada penderita Waldenstrom’s Macroglobulinemia: laporan kasus. Intisari Sains Medis, 10(2). https://doi.org/10.15562/ism.v10i2.375
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Alamsyah Alamsyah
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ISM Journal

Herniah Asti Wulanjani
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