Successful renal transplantation to a recipient with type II cryoglobulinemia: a case report.

BACKGROUND: Recurrence of glomerulonephritis is an important risk factor for renal graft dysfunction. Cryoglobulinemia is known as a relatively rare cause of renal failure, and doctors are usually hesitant to perform transplantation on a recipient with cryoglobulinemia because of the risk for graft loss. We present a case of renal transplantation on a patient with organ manifestations of type II cryoglobulinemia. CASE PRESENTATION: At the age of 44 years, the patient developed acute kidney injury and purpura on the lower extremities with type II cryoglobulinemia after interferon therapy for hepatitis C virus. Cryoglobulinemic glomerulonephritis was suspected; however, despite immunosuppressive therapy combined with plasmapheresis, she eventually needed hemodialysis treatment. She was referred to us at the age of 49 years for renal transplantation. Cryocrit was 14% and the organ manifestations persisted, including the lower extremity purpura and neurologic symptoms. After monitoring and confirming sufficient suppression of cryoglobulin concentration by immunosuppressive treatment with prednisolone, cyclophosphamide, and rituximab combined with plasmapheresis, the operation was performed. After transplantation, the cryoglobulin concentration was continuously monitored, and plasmapheresis and rituximab infusion were performed as appropriate. Her graft function has remained stable for 2 years and 6 months. CONCLUSION: Our case suggested that a patient with cryoglobulinemia and persistent organ manifestations can receive a renal graft if the cryoglobulin concentration is sufficiently controlled by pretransplant treatment.

Normothermic Cardiopulmonary Bypass in Patient With Waldenström's Macroglobulinemia and Cryoglobulinemia: A Case Report.

Waldenström's macroglobulinemia (WM) manifests as hyperviscosity syndrome. Cryoglobulinemia, which may increase blood viscosity or induce thrombosis in association with decreased body temperature, can occur in combination with WM. We describe the management of an 82-year-old woman with WM, hyperviscosity syndrome, and cryoglobulinemia who required open aortic valve replacement. Decreased body temperature in this patient was prevented during cardiopulmonary bypass by using a forced air warming system and normothermic cardioplegia with continuous warm blood cardioplegia perfusion.

Pattern of primary vasculitis with peripheral ischemic manifestations: report of a case series and role of vascular surgery.

AIM OF THE WORK: The aim of the present work was to study the role of vascular surgery in the management of primary vasculitis patients with peripheral ischemic manifestations. PATIENTS AND METHODS: Ten primary vasculitis patients with peripheral ischemic manifestations were studied and reviewed for the diagnosis, clinical manifestations, investigations, treatment options and role of vascular surgery. The Birmingham Vasculitis Activity Score (BVAS) was recorded. RESULTS: Giant cell arteritis was present in one patient; granulomatosis with polyangiitis in 5, essential cryoglobulinemic vasculitis in 3 and 1 (child) had Henoch-Schönlein purpura. They showed the following peripheral vascular manifestations: intermittent claudications, Raynauds, deep venous thrombosis and thrombophelebitis in 10% each; digital ulceration and trophic changes in 20% while acrocyanosis and dry gangrene were present in 30%. Renal involvement was present in 60% of patients. The mean BVAS was 11.5 ± 6.57 at initial presentation. The disease activity remarkably improved over the disease course in all patients to be at their last visit (2.6 ± 2.22) (p=0.002). Regarding the vascular surgery role in their management, in addition to their medical treatment, 40% required an additional surgical intervention. Two had a minor amputation of the toes; one performed thoracoscopic cervical sympathectomy and another needed tibial angioplasty. CONCLUSION: Primary vasculitis patients presenting with peripheral ischemic manifestations require surgical attention. Their management is essentially medical and individualized to the diagnosis and presenting symptoms. Endovascular treatment may offer a safe and less invasive approach in high surgical risk patients. Sympathectomy is of high therapeutic potential in those with severe pain and trophic changes.

Cryoglobulinemia with acronecrosis not associated with hepatitis C infection: a case report.

Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation.

Development of acute inflammatory arthritis by granulocyte-macrophage colony-stimulating factor during autologous stem cell transplantation for cryoglobulinemia.

Hematopoietic growth factors (HGFs) are now known to influence the function of mature myeloid cells as well as their traditional roles as regulators of hematopoiesis. It became apparent that they could take part in inflammatory and immune responses by activating monocytes/macrophages to release mediators of such responses. We describe a 53-year-old male who developed acute inflammatory arthritis by granulocyte-macrophage colony-stimulating factor (GM-CSF) following autologous stem cell transplantation for cryoglobulinemia. Arthritis improved dramatically soon after GM-CSF was withdrawn and steroid therapy was admitted. This case may lead to further attention for this potential problem since HGFs are frequently used in a variety of clinical settings.

[Symmetrical peripheral gangrene of unknown etiology]. / Symmetrische periphere Gangrän unbekannter Atiologie.

We describe the case of a 19-year-old female patient who presented with acute symmetrical peripheral gangrene (SPG), including all fingers and toes. Further diagnostic work-up did not reveal any underlying condition other than a non-specific transient cryoglobulinemia. Despite immediate anticoagulopathic and anti-inflammatory treatment, all fingers and toes had to be amputated.

Laparoscopic splenectomy for hematologic diseases.

BACKGROUND: Laparoscopic splenectomy has been demonstrated to be technically feasible and safe for the treatment of hematologic diseases. METHODS: The study comprised 64 consecutive patients with chronic immune thrombocytopenia purpura (ITP) who were admitted to our hospital from 1992 to 2000 and underwent laparoscopic splenectomy. Forty-one consecutive patients with ITP who underwent open splenectomy performed at the same hospital by the same surgical team between 1986 and 2000 were selected as the control group. RESULTS: We performed laparoscopic splenectomies for ITP, hereditary spherocytosis (HS), malignant lymphoma, autoimmune hemolytic anemia, and cryoglobulinemia. Laparoscopic surgery as compared with open surgery in ITP revealed a significantly lower pain medication requirement, an earlier resumption of oral intake, and a shorter hospital stay, but a longer operative time. During the present study (range: 3.8-80 months), the cumulative rate of nonrecurrence was 67.9% at 5 years after surgery, which was similar to the rate for the previous open splenectomy. CONCLUSIONS: Laparoscopic splenectomy is considered to be a suitable alternative therapeutic modality in the treatment of hematologic diseases.

Excimer phototherapeutic keratectomy for corneal subepithelial cryoglobulin deposits.

A 64-year-old man with a history of cryoglobulinemia and repeated scraping of immunoglobulin deposits from both superficial corneas presented with gradual decrease in vision and progressive hyperopia caused by circumferential redeposition of the cryoglobulins in his corneas. He had debridment of the deposits combined with phototherapeutic keratectomy to smooth the corneal surface. Visual acuity and reading capacity markedly improved, and hyperopia regressed by 5.75 diopters (D) in the right eye and 5.25 D in the left. Follow-up was 3 years.

Type 1 cryoglobulinaemia associated with a thymic tumour: successful treatment with plasma exchange.

Thymic tumours are associated with a wide range of autoimmune and haematological disorders, notably myasthenia gravis, red cell aplasia, and systemic lupus erythematosus. An association with cryoglobulinaemia has only once been reported previously. In this report we describe a 60-yr-old male patient with a spindle cell thymoma, treated surgically, who also had type 1 cryoglobulinaemia, with severe peripheral circulatory impairment with digital ulceration and a mononeutitis multiplex. The patient has been successfully treated with prednisolone, immunosuppression and plasma exchange.

Treatment of life-threatening autoimmune diseases with myeloablative doses of immunosuppressive agents: experimental background and rationale for ABMT.

We describe a case of autoimmune-mediated mixed cryoglobulinemia with vasculitis and nephritis that was successfully treated with acute myeloablative immunosuppressive treatment. The case suggests that elimination of autoreactive lymphocytes followed by de novo regeneration of lymphocytes from autologous stem cells that re-acquire unresponsiveness towards self may be successfully accomplished in life-threatening autoimmune diseases. The combination of acute myeloablative immunosuppression followed by syngeneic stem cell transplantation may be an effective treatment for severe autoimmune diseases.