Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature.

Human Parvovirus B19 (PV B19) is one of the several recently described 'emerging viruses' and has been identified as the etiological agent of 'fifth disease' in childhood. Human PV B19, which is the etiological agent of transient erythroblastopenia in hemolytic anemia, is also a recognized rare cause of red cell aplasia in immunocompromised patients, including transplant recipients. To date, 26 cases of PV B19-induced red cell aplasia have been reported in solid organ transplant recipients. Twelve patients had cyclosporine-based immunosuppression and 14 had tacrolimus-based immunosuppression. Sixteen of these patients required treatment with commercial intravenous immunoglobulin alone, 1 required treatment with intravenous immunoglobulin and plasmapheresis, 4 required intravenous immunoglobulin and erythropoietin, 1 required treatment with intravenous immunoglobulin and conversion of tacrolimus to cyclosporine, 1 had improvement in hematocrit with erythropoietin alone and in 3 patients the disease was self-limiting. Herein, we report a case of pure red cell aplasia caused by acute PV B19 infection in a renal transplant recipient in whom the immunosuppressive regimen included prednisone, mycophenolate mofetil and tacrolimus and the red cell aplasia resolved with discontinuation of mycophenolate mofetil.

Longitudinal study of chronic sarcoidosis with low-dose maintenance corticosteroid therapy. Outcome and complications.

A series of 181 patients with chronic persistent sarcoidosis for more than 5 years have been reviewed, with a mean follow-up period of 14 years, and 77% followed for more than 10 years. Efforts to identify more white patients meeting the criteria of such persistent disease were unsuccessful. Seventy-eight percent were black, and 69% were women. Pulmonary disease was the major manifestation in 70% and was present in 88%. The early major disease feature predicts the nature of the long-term manifestation in 94%. Ninety-four percent were treated with prednisone, with 63% for more than 5 years. Fifty-three percent required continued treatment, and of those receiving continued treatment, 91% were maintained on doses of no more than 15 mg daily and 65% on no more than 10 mg of prednisone daily. Relapses were frequent as prednisone was withdrawn, occurring at least once in 75% and several times in 51%. Low-dose prednisone treatment with 5-15 mg daily provides significant benefit. A mean treatment period of 8 years was observed (range: 10 days to 24 years). The benefits of treatment greatly exceed the infrequent complications. In Baltimore, chronic sarcoidosis with persistent disease seems to be more frequently encountered in black patients than in white patients.

Diagnosis and management of retroperitoneal hematomas after femoral vein cannulation for hemodialysis.

Six patients were identified who suffered retroperitoneal hemorrhage during or after hemodialysis via a femoral vein approach. Hypotension developing during dialysis or lower quadrant abdominal pain was the common presenting symptom. All patients had decreasing hematocrits and five of the six patients required blood transfusions. The method of diagnosis was clinical, with plain abdominal x-ray examination and cystography being the most helpful adjuncts. All patients were successfully treated with volume resuscitation and removal of the femoral vein catheters; none required operative intervention or suffered adverse late effects. Femoral vein dialysis is useful for uremic patients without other peripheral vascular access options, and its associated morbidity may be minimized provided complications are recognized and promptly treated.

APUD system neoplasms in renal transplant patients.

Apudomas are uncommon neoplasms composed of neuroendocrine cells. They include carcinoid tumors, islet cell tumors, and small cell lung carcinoma. We found six cases of apudomas in a series of 1028 renal transplants from three medical centers (0.58%). One of these had been reported in 1976. The cases included a carcinoid tumor of a Meckel's diverticulum discovered and removed prior to transplantation, with no evidence of recurrence 9 years later. A small cell lung carcinoma was discovered 40 months after renal transplantation, with a fatal outcome 6 months later. Four clinically occult apudomas were found at autopsy, including one gastric and one bronchial carcinoid tumor, one multicentric pancreatic islet cell neoplasm, and one case of multiple ileal carcinoids. With the exception of the small cell lung cancer, none of the apudomas was clinically significant, and none was associated with carcinoid or other paraneoplastic syndrome. These cases illustrate the difficulty of diagnosis of apudomas in patients with renal failure and the usually benign nature of these tumors despite the administration of potent immunosuppressive agents.

Renal transplantation in a patient with multiple myeloma and light chain nephropathy.

A case of multiple myeloma in a 41-year-old white man that resulted in chronic renal failure is discussed. During the period of hemodialysis treatment, remission of the patient's myeloma was induced by chemotherapy. Thereafter a transplanted cadaver kidney functioned well for 3.5 years despite episodes of sepsis, administration of nephrotoxic chemotherapeutic agents, and recurrence of the myeloma with intermittent excretion of Bence Jones protein in the urine. The results of this fully documented case, as well as two other cases we have previously reported, support the strategy of offering cadaver renal transplantation to carefully selected individuals who require long-term dialysis and whose myeloma is in remission after chemotherapy.

The longitudinal study of chronic sarcoidosis.

Although spontaneous remissions are frequent in sarcoidosis, chronic persistent disabling disease is also observed. This feature seems more frequent and more severe in black patients, as has been previously reported (10). The more extensive and severe the initial disease manifestations, the more likely it is that the disease will continue. Corticosteroids are usually beneficial and complications are infrequent. Delayed or interrupted treatment seems to allow progression or irreversible disease or both. Patient compliance is an obvious important factor. Relapses are frequent as treatment is withdrawn but are usually at least partially reversible. Some deterioration is observed with repeated relapses. Low dose daily prednisone (5-15 mg) seems to prevent relapses. Patient compliance is facilitated with daily treatment rather than alternate day therapy. Prolonged treatment for 10 to 15 or more years is often required. Chloroquine is particularly helpful in skin and mucosal disease. The necessity for long-term thoughtful management is obvious.

Skin autografts to cover exposed renal allografts.

Exteriorized renal allografts that protrude abnormally after dehiscence of transplant wounds pose a rare but formidable surgical problem. If good renal allograft function is present and wound sepsis is controlled, the renal allograft need not be removed even if the wound cannot be approximated. The exposed kidney behaves like other body soft tissues, and resurfacing the renal cortex with skin autografts can be performed successfully. We present four cases demonstrating that split-thickness autografts can be successfully utilized to cover an exposed renal allograft with salvage of allograft function. The incidence of this situation was 0.62% in a series of 641 consecutive renal transplants. We also observed, incidentally, that there was no apparent linkage between the HL-A complex and the tendency for keloid formation.

Painful crises following renal transplantation in sickle cell anemia.

A 34 year old woman with sickle cell (SS) anemia and chronic renal failure of unknown etiology was maintained on dialysis for 11 months before she received a cadaveric renal transplant. After 24 months, transplant function is excellent although a mild urinary concentrating defect (Umax = 532 mOsm/liter) is present. Renal biopsies five and 11 months after transplant revealed mild focal interstitial infiltrates and mesangial thickening. A major complication has been the reemergence of numerous severe painful crises, inferred to be caused by an increased blood viscosity consequent to a rising hematocrit value, after a hiatus of many years. The succession of crises was stopped with a prophylactic partial exchange transfusion program, reemerged when the program was discontinued, and was stopped again when the transfusion program was reinstituted. We conclude that renal transplantation may be successfully performed in patients with sickle cell disease. Complications of the hemoglobinopathy may develop, but painful crises can be successfully managed with judicious transfusion therapy.

Mycotic aneurysm with recurrent sepsis complicating post-transplant nephrectomy.

A hypertensive 37-year-old man underwent renal transplantation. The transplanted kidney was removed 5 days later, 21 months before his death. He was maintained after transplantation by hemodialysis. His postoperative course was complicated by recurrent pseudomonal infection with bacteremia. He presented with symptoms of acute peritoneal irritation and hypovolemia followed by sudden death. Postmortem examination revealed that a saccular mycotic aneurysm of the left common iliac artery, which was the site of post-transplant nephrectomy, had ruptured into the peritoneal cavity. The case exemplifies postoperative mycotic aneurysm, the frequently obscure clinical findings of iliac artery aneurysms, and the need for a high index of suspicion of mycotic aneurysm in patients undergoing renal transplantation.

Perfusion nephropathy in human transplants.

To determine whether perfusion preservation affected the structure and survival of kidney transplants, we correlated clinical and histologic data in 77 kidneys biopsied one hour after transplantation. Twenty-one of 36 perfusion-preserved kidneys had a glomerular capillary lesion suggestive of intravascular coagulation. None of 41 kidneys preserved by hypothermia alone had this lesion. Presence of the lesion did not correlate with donor or recipient characteristics, warm or cold ischemia time, HLA match, percentage of preformed lymphocytotoxic antibody titers or perfusion characteristics. Of 21 transplants with the lesion, nine required nephrectomy by one month, and one-month serum creatinine was less than 2.0 mg per deciliter in only three of the remaining 12 transplants. We conclude that perfusion preservation may cause pathologic changes that may adversely affect kidney-transplant function. The causes of the pathologic process remain unclear.

Extended experience in the long-term corticosteroid treatment of pulmonary sarcoidosis.

A 15-yr experience with symptomatic pulmonary parenchymal sarcoidosis treated with corticosteroids has included 250 patients. Within the past 5 yr 192 patients have been actively studied and these are reported in detail. Varied clinical observations are described. Serial measurements of vital capacity and steady-state carbon monoxide diffusing capacity were performed. All of these patients were sufficiently symptomatic and incapacitated to require treatment. Ninety-one percent were Black, 72% were female, and 72% were in the third and fourth decades. In response to treatment there was overall clinical improvement and an increase in vital capacity in 150 (83%) of 180 patients, although only a temporary increase in 12 of these patients. By contrast, only 82 (46%) showed an increase in carbon monoxide steady-state diffusing capacity and only temporarily in 19 patients. Changes in vital capacity correlate well with changes in symptoms and the radiographic appearance and are of great assistance in assessing the clinical course and need for continued treatment. The diffusing capacity is more difficult to interpret. Continued treatment was required in 74 patients (48%) because of recurrent clinical relapses as treatment was withdrawn. Such relapses were observed in 70% of the patients. Improvement and discontinuation of treatment was achieved in 66 patients (42%). In the 44 in whom there was no observed relapse, 34 were treated for less than 2.5 yr. In the total group, longer periods of treatment, 2.5-10 yr or more, were required in 104 patients (54%). In this series of 192 patients there were 7 deaths, with 22 deaths in the total 15-yr series of 250 patients. Of these, 18 were directly attributed to the sarcoidosis. Clinical improvement coincident with corticosteroid treatment and the frequency of relapse as treatment is withdrawn strongly support the benefits of corticosteroid treatment for symptomatic pulmonary sarcoidosis. When relapses occur and recur, retreatment is necessary and often long-term maintenance doses of prednisone (10-15 mg daily) are required for many years. The relatively infrequent problems related to this treatment are greatly exceeded by the clinical benefits.

Renal transplantation in multiple myeloma. A case report.

A patient with plasma cell myeloma presented in severe renal failure but was otherwise considered a good risk candidate for chemotherapy. Supported by hemodialysis she received intensive cyclophosphamide chemotherapy, resulting in a stabilization of all overt manifestations of her disease except for the renal failure. After 11 1/2 months of follow-up without showing any further progression of the myeloma process, she received a successful cadaveric renal transplant, which functioned well until the time of her death 15 months later. No evidence of recurrent myeloma kidney disease was found at postmortem examination.