Recurrent, Isolated Factor X Deficiency in Myeloma: Repeated Normalization of Factor X Levels after Cytostatic Chemotherapy Followed by Late Treatment Failure Associated with the Development of Systemic Amyloidosis

I Schwarzinger; M Stain-Kos; R Bettelheim; I Pabinger; P Kyrle; P Kalhs; S Kapiotis; U Jäger; K Lechner

doi:10.1055/s-0038-1646337

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1992; 68(06): 648-651
DOI: 10.1055/s-0038-1646337

Original Article

Schattauer GmbH Stuttgart

Recurrent, Isolated Factor X Deficiency in Myeloma: Repeated Normalization of Factor X Levels after Cytostatic Chemotherapy Followed by Late Treatment Failure Associated with the Development of Systemic Amyloidosis

I Schwarzinger

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

M Stain-Kos

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

R Bettelheim

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

I Pabinger

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

P Kyrle

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

P Kalhs

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

S Kapiotis

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

U Jäger

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

,

K Lechner

The 1st Medical Department, Division of Hematology and Blood Coagulation, University of Vienna, Austria

› Author Affiliations

Abstract

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Summary

We describe the case of a 64-year-old woman with isolated severe factor X deficiency associated with kappa light chain myeloma. At the time of diagnosis there was no evidence for amyloidosis. Complete remission (CR) of myeloma as well as normalization of factor X levels were achieved after cytostatic chemotherapy. Subsequently, factor X deficiency recurred twice without any evidence for relapse of myeloma. The first time factor X normalized again following cytostatic treatment, the second time, however, factor X deficiency was refractory to chemotherapy. Finally, relapse of myeloma became evident associated with rapidly progressing, systemic amyloidosis, which was fatal within a few months. Initially, factor X infusion studies showed a normal recovery, but when amyloidosis became overt the recovery decreased to 0%. We assume that factor X deficiency was due to a binding of factor X to kappa light chains associated with the proliferation of the malignant myeloma cell clone.

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References

REFERENCES
1 Josso F, Boussen M, Prou-Wartelle O, Piguet H, Sultan Y, Bousser J. Anomalies complexes de la coagulation dans deux cas de maladie de Kahler avec syndrome hémorragique. Signification du deficit en facteur Stuart au cours du myelome. Nouv Rev Fr Hematol 1966; 6: 739-744
2 Perkins HA, MacKenzie MR, Fudenberg HH. Hemostatic defects in dysproteinemias. Blood 1970; 35: 695-707
3 Greipp PR, Kyle RA, Bowie EJW. Factor-X deficiency in amyloidosis: a critical review. Am J Hematol 1981; 11: 443-450
4 Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc 1983; 58: 665-683
5 Korsan-Bengtsen K, Hjort PF, Ygge J. Acquired factor X deficiency in a patient with amyloidosis. Thromb Diath Haemorrh 1962; 7: 558-566
6 Howell M. Acquired factor X deficiency associated with systematized amyloidosis - a report of a case. Blood 1963; 21: 739-743
7 Pechet L, Kastrul JJ. Amyloidosis associated with factor X (Stuart) deficiency. Case report. Ann Intern Med 1964; 61: 315-318
8 Galbraith PA, Sharma N, Parker WL, Kilgour JM. Acquired factor X deficiency. Altered plasma antithrombin activity and association with amyloidosis. JAMA 1974; 230: 1658-1660
9 Furie B, Greene E, Furie BC. Syndrome of acquired factor X deficiency and systemic amyloidosis. In vivo studies of the metabolic fate of factor X. N Engl J Med 1977; 297: 81-85
10 Greipp PR, Kyle RA, Bowie EJW. Factor X deficiency in primary amyloidosis. Resolution after splenectomy. N Engl J Med 1979; 301: 1050-1051
11 Cohen D, Pras M, Franklin EC, Frangione B. Characterization of amyloid deposits and p component from a patient with factor X deficiency reveals proteins derived from a lambda VI light chain. Am J Med 1983; 74: 513-518
12 Camoriano JK, Greipp PR, Bayer GK, Bowie EJW. Resolution of acquired factor X deficiency and amyloidosis with melphalan and prednisone therapy. N Engl J Med 1987; 316: 1133-1135
13 Furie B, Voo L, McAdam KPWJ, Furie BC. Mechanism of factor X deficiency in systemic amyloidosis. N Engl J Med 1981; 304: 827-830
14 Case JR DC, Sonneborn HL, Paul SD, Hayes DM, Dorsk RJ, Carroll RJ, Bove L. Combination chemotherapy for multiple myeloma with BCNU, Cyclophosphamide, Vincristine, Melphalan, and Prednisone (M2-Protocol). Oncology 1985; 42: 137-140
15 Lechner K. Gerinnungstests: Bestimmung der Aktivität oder Konzentration der Gerinnungsfaktoren. In: Blutgerinnungsstörungen. Springer, Berlin: 1982. pp 178-209
16 Vigilano EM, Horowitz HI. Bleeding syndrome in a patient with IgA myeloma: Interaction of protein and connective tissue. Blood 1967; 29: 823-836
17 Richard C, Cuadrado MA, Prieto M, Batlle J, Fernandez MFL, Salazar MLR, Bello C, Recio M, Santoro MT, Casares MTG, Zubizarreta A. Acquired von Willebrand disease in multiple myeloma secondary to absorption of von Willebrand factor by plasma cells. Am J Hematol 1990; 35: 114-117
18 Conley L, Hartmann RCA. A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus. J Clin Invest 1952; 31: 621-622
19 Buxbaum JN, Hurley ME, Chuba J, Spiro T. Amyloidosis of the AL type. Clinical, morphologic and biochemical aspects of the response to therapy with alkylating agents and prednisone. Am J Med 1979; 67: 867-878
20 Fielder K, Durie BGM. Primary amyloidosis associated with multiple myeloma. Predictors of successful therapy. Am J Med 1986; 80: 413-418
21 Gertz MA, Kyle RA. Reponse of primary hepatic amyloidosis to melphalan and prednisone: a case report and review of the literature. Mayo Clin Proc 1986; 61: 218-223
22 Schattner A, Varon D, Green L, Hurwitz N, Bentwich Z. Primary amyloidosis with unusual bone involvement: reversibility with melphalan, prednisone, and colchicine. Am J Med 1989; 86: 347-348
23 Stone MJ, Frenkel EP. The clinical spectrum of light chain myeloma. A study of 35 patients with special reference to the occurrence of amyloidosis. Am J Med 1975; 58: 601-619
24 Stone MJ. Amyloidosis: a final common pathway for protein deposition in tissues. Blood 1990; 75: 531-545