ASH Image Bank (2001); doi:10.1182/ashimagebank-2001-100185
Copyright © 2001 by the American Society of Hematology.
Multiple Myeloma: Platelet Dysfunction
John Lazarchick, M.D.
Medical University of South Carolina
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Figure 4. Bone marrow aspirate with sheet of plasma cells. Dutcher bodies are evident in a number of these cells.
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Figure 6. Immunohistochemical stain of bone marrow biopsy with antibody to kappa light chain shows the plasma cells to be negative.
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Figure 7. Serum protein electrophoresis showing a monoclonal band in the beta region. Immunofixation studies showed this to be an IgA monoclonal protein.
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Figure 9. Panel A: Flow cytometric analysis on the bone marrow aspirate for cytoplasmic light chain restrcition using CD38 to isolate on the plasma cells. Panel B shows the CD38+ cells to mark for Lambda light chain. Panel C: The CD38+ cells are negative for Kappa light chain.
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Figure 10. Platelet aggregometry results with adenosine diphosphate (ADP) and arachidonic acid agonists are illustrated. The patient has a blunted initial aggregation response to ADP with minimal ATP release and then disaggregation. (Panel A and Panel B: patient = blue;control= pink).
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Figure 11. Skull x-ray showing multiple lytic areas. Compression fracture was also noted at T7 (not shown).
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Clinical Summary
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A 54-year-old African American man was brought to the emergency department after falling in his bathroom and being unable to walk. He stated that his right leg gave out right before the fall. He also stated he has pain in his mid-back, but that this was present before the fall. He gave a smoking history of 20 pack years. X-rays in the emergency department showed lytic pelvic lesions and compression fracture of T7. He was admitted for further evaluation. The patient was seen in consultation by the Hematology Service and diagnosed with IgA myeloma, stage IIIA, and is receiving chemotherapy.
Because of the prolonged bleeding time noted as part of his evaluation for excessive oozing from his marrow biopsy site, platelet function studies were performed.
Lab: Hemoglobin (Hgb) 12.9, white blood cells (WBC) 6090/uL, platelets 210,000/uL; total protein 10.4g/dL, albumin 2.8 g/dL and IgG 7.6 g/dL.( IgG 410, IgM 18 and IgA 6.98g/uL); creatinine 1.95; beta 2 microglobulin 2.3; calcium 9.4. MRI compression fracture at T7.
Sex
Male
Age
54
Ethnicity
African American
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Diagnosis
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IgG or IgA myeloma,Waldenstrom's macroglobulinemia, Monoclonal gammopathy of unknown significance (MGUS).
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Discussion
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The hemorrhagic diathesis that occurs in patients with plasma cell dyscrasias can be attributed to multiple mechanisms. Among these are mild to moderate thrombocytopenia due to marrow replacement by tumor, hypoplasia secondary to chemotherapy, an acquired platelet dysfunction due to monoclonal protein directly or indirectly (azotemia), defects in the micro-vasculature from amyloid deposition, the monoclonal protein having inhibitory activity to specific coagulation factors, and to the development of a hyperviscosity syndrome. Bleeding occurs in approximately 15% of patients with IgG myeloma and in more than 30% of patients with IgA myeloma.
Thrombocytopenia can result from extensive marrow replacement by plasma cells but platelet counts are seldom less than 50,000/uL and in most patients the count is normal or only mildly decreased. A qualitative platelet defect can be demonstrated in approximately one-third of patients with IgA myeloma or Waldenstrom's macroglobulinemia and in 15% of patients with IgG myeloma. Inhibition of platelet function usually correlates with the serum concentration of the paraprotein. Defects in aggregation and adenosine triphosphate (ATP) release are readily demonstrated using platelet aggregometry. The pattern is most often that of an acquired storage pool defect with blunted aggregation and diminished ATP release. Treatment of acute bleeding episodes may require plasma- pheresis. Treatment of chronic bleeding can be managed with platelet transfusions and chemotherapy for the myeloma.
Other hemostatic defects which can be seen in this patient group include factors X and IX deficiencies due to adsorption by amyloid deposits in the microvasculature, paraproteins with anti-factor VIII inhibitory activity or circulating heparin-like activity, and impaired fibrin monomer polymerization. Rarely these paraproteins will cause an acquired von Willebrand syndrome or systemic fibrinolysis.
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Differential Diagnosis
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Monoclonal gammopathy of unknown significance (MGUS).
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| Related ASH-SAP Chapter: |
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Chapter 13: Plasma cell dyscrasias
Copyright © 2001 by the American Society of Hematology.
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Clinical Summary
Diagnosis
