Lab Med Online.  2013 Jul;3(3):183-188. 10.3343/lmo.2013.3.3.183.

Bleeding Tendency of a Light Chain (AL) Amyloidosis Patient Accompanied by Asymptomatic Plasma Cell Myeloma

• Lee HJ
• Kim JE
• Park SY
• Kim HK

• KMID: 2053555
• DOI: http://doi.org/10.3343/lmo.2013.3.3.183

Abstract

We present a case of abrupt-onset hemorrhagic tendency in a patient with amyloidosis who also had asymptomatic plasma cell myeloma. The patient was a 66-yr-old man with no previous history of hemorrhagic tendency and no family history of hemorrhagic disease. On examination, the prothrombin time and activated partial thromboplastin time were found to be prolonged and were not corrected even after a mixing test; moreover, the levels of coagulation factors I, II, V, VII, and X were almost normal. We therefore considered the presence of a nonspecific coagulation inhibitor. Although the von Willebrand factor (vWF) activity and vWF antigen level were normal due to sampling following transfusion, the increased closure time on PFA-100 (Siemens) analysis and the absence of ristocetin-induced platelet aggregation suggested the presence of acquired von Willebrand syndrome (vWS). After chemotherapy, the patient showed alleviation in the bleeding symptoms. Therefore, testing for acquired vWS should be considered when a patient has a history of recent bleeding with underlying amyloidosis.

Keyword

Amyloidosis; Plasma cell myeloma; Hemorrhagic tendency; Acquired von Willebrand syndrome

MeSH Terms

Amyloidosis
Blood Coagulation Factors
Hemorrhage
Humans
Light
Multiple Myeloma
Partial Thromboplastin Time
Plasma
Plasma Cells
Platelet Aggregation
Prothrombin Time
von Willebrand Factor
Blood Coagulation Factors
von Willebrand Factor

Figure

• 

  Fig. 1 Abdominal computed tomography showed splenic subcapsular hematoma (red arrow). Spleen showed increase in size, lobulating contour, decreased perfusion with delayed capsular enhancement (green arrow) on portal phase and increased amount of perisplenic fluid.

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  Fig. 2 (A) Bone marrow aspiration showed increased number of plasma cells (21.8% of total nucleated cells, Wright Giemsa, ×400). (B) Bone marrow biopsy showed interstitial deposits of pink amorphous waxy-appearing substances (H&E, ×200). (C, D) Increased clonal plasma cells are negative for cytoplasmic kappa light chain (C) and positive for lambda light chain (D) in bone marrow biopsy (Immunohistochemical stain, ×400). (E, F) Congo red stain produced amyloid pink to red (E) by standard light microscopy and apple-green birefringence (F) under polarized light microscopy (Congo Red, ×400).

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  Fig. 3 Results of platelet aggregation test in the patient (A) and a healthy control (B). The patient showed normal aggregation response to 10 µM ADP (71%), 10 µM epinephrine (75%) and 2 µg/mL collagen (72%), but no aggregation response to 1.25 mg/mL ristocetin.

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