deamino-arginine-vasopressin and Multiple-Myeloma

Autologous stem cell transplant has been shown to prolong survival in multiple myeloma (MM). A common complication of the pre-transplant conditioning chemotherapy is severe multi-lineage cytopenias, resulting in significant transfusion requirements. Jehovah's Witnesses are members of a religious group that do not accept the transfusion of blood products. Many large transplant centers refuse to perform transplantation in Jehovah's Witnesses due to the complexity of treating cytopenic patients without blood product transfusions. However, some transplant centers that specialize in "bloodless" medicine and surgery have successfully transplanted in Jehovah's Witnesses without transfusion support.. In order to maximize successful outcomes in this population, potential transplant candidates are treated with a variety of agents to maximize baseline hemoglobin and platelet counts. In preparation for the first two "bloodless" transplants for MM at our institution, we conducted a retrospective study of patients with MM who underwent a transplant in the preceding year.. Of the 60 patients reviewed, only six required packed red blood cell transfusion, whereas 39 required at least one platelet transfusion. These findings helped us to design a novel protocol for a "bloodless" autologous transplant. We administered romiplostim, a thrombopoietin (TPO) agonist, along with aminocaproic acid, desmopressin, and vitamin K post-transplant to two Jehovah's Witness patients to mitigate the risk of thrombocytopenia. Neither patient experienced significant bleeding nor qualified for platelet transfusion, and underwent successful and uncomplicated transplantation.. We propose that the use of romiplostim or similar TPO agonists can be used to maximize the chance of a successful "bloodless" transplant for stem cell recipients.

Topics: Adult; Aged; Aminocaproic Acid; Autografts; Deamino Arginine Vasopressin; Female; Humans; Jehovah's Witnesses; Male; Middle Aged; Multiple Myeloma; Receptors, Fc; Recombinant Fusion Proteins; Stem Cell Transplantation; Thrombopoietin; Vitamin K

A case of acquired von Willebrand disease (AvWD) associated with an IgA lambda multiple myeloma is reported. No form of inhibitor could be detected. SDS-agarose gel electrophoresis patterns of von Willebrand factor (vWF) both in plasma and platelet lysates were normal but a decrease in all-sized multimers with a type IA pattern was seen. After 1-deamino-8-D arginine vasopressin (DDAVP) infusion, vWF multimers larger than those seen in the resting state appeared in patient plasma, which were progressively cleared. Indirect immunofluorescence studies with a monoclonal antibody to vWF showed that vWF was selectively absorbed into myelomatous cells. This is the first case of AvWD associated with multiple myeloma resulting from the selective absorption of vWF into abnormal plasma cells. This feature established a new pathophysiological mechanism of AvWD in multiple myeloma and probably in other lymphoproliferative diseases.

Topics: Absorption; Aged; Deamino Arginine Vasopressin; Electrophoresis, Agar Gel; Female; Fluorescent Antibody Technique; Humans; Multiple Myeloma; Plasma Cells; von Willebrand Diseases; von Willebrand Factor

The response to a single intravenous infusion of 1-deamino-8-D-arginine vasopressin (DDAVP, desmopressin) was studied in two patients with acquired von Willebrand syndrome associated with IgG-kappa myeloma. Following infusion of DDAVP (0.3-0.4 micrograms/kg), prolonged bleeding time was normalized; plasma ristocetin cofactor activity, von Willebrand factor antigen, and factor VIII activity were remarkably increased; and high-molecular-weight forms of von Willebrand factor were demonstrated by crossed immunoelectrophoresis in both patients. Excellent hemostasis was achieved following administration of DDAVP in one patient when it was used for the treatment of gum bleeding and for the prophylaxis of bleeding during and after dental extractions. These observations suggest that DDAVP is an effective alternative to blood products for at least some patients with acquired von Willebrand syndrome in addition to patients with inherited von Willebrand disease, hemophilia A, and uremia.

Topics: Blood Coagulation Tests; Deamino Arginine Vasopressin; Female; Hemostasis, Surgical; Humans; Immunoglobulin G; Immunoglobulin kappa-Chains; Infusions, Parenteral; Middle Aged; Multiple Myeloma; von Willebrand Diseases