Hemoglobinuria paroxística nocturna / Paroxysmal nocturnal hemoglobinuria

ResumenLa hemoglobinuria paroxística nocturna es una anemia hemolítica crónica, adquirida, poco común, que afecta con igual frecuencia ambos sexos. Se manifiesta a cualquier edad y con mayor incidencia en países del sudeste asiático. Es el resultado de la expansión clonal no maligna de células progenitoras hematopoyéticas. Se caracteriza por anemia hemolítica intravascular, tendencia a la trombosis y un componente variable de insuficiencia medular.Se asocia a otras patologías hematológicas como anemia aplásica y síndrome mielodisplásico. La citometría de flujo es el método de elección para diagnóstico. El eculizumab y el trasplante de médula ósea alogénico son las únicas terapias efectivas.

Abstract:Paroxysmal nocturnal hemoglobinuria is a rare acquired chronic hemolytic anemia, which affects both sexes with equal frequency. It occurs at any age and more frequently in Southeast Asian countries. It is the result of non malignant clonal expansion of hematopoietic progenitor cells. It is characterized by intravascular hemolytic anemia, recurrent thrombosis and a variable component of bone marrow failure. It is associated with other hematologic disorders such as aplastic anemia and myelodysplastic syndrome. Flow cytometry is the method of choice for diagnosis. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies.

Myoglobinuria: evaluation of methods in the clinical diagnosis acute renal failure.

Patients with red--brown urine which may be a sign of myoglobinuria, can develop acute renal failure. We assayed serum creatinine, blood urea nitrogen (BUN) and creatine kinase activity in a total of 33 patients of equal groups, A (automobile accident), B (trauma) and C (undergoing rhabdomyolysis). In addition we tested 132 urine samples for the presence of myoglobin using a dipstick assay. Only five patients in group A showed any sign of myoglobinuria with increased creatine kinase activity upto 7 times the normal value but their serum creatinine level and BUN were within the normal range. In contrast, all 22 patients in group B and C showed myoglobinuria and above normal concentrations of serum creatinine and BUN, with significantly increased (p < 0.0001) creatine kinase activity upto 150 times the normal range. Four of the most seriously ill patients in group C developed acute renal failure. Supplementation of routine determinations of serum creatinine and BUN and serum creatine kinase activity with a rapid test for myoglobinuria provides an extra indication of impending renal dysfunction. It may be beneficial in the emergency management of these patients.

Exogenous causes of myoglobinuria: review of 26 cases

In this article, I review various causes of exogenous myoglobinuria(MU) and its pathogenesis in 26 consecutive patients admitted to emergency room, Asan Medical Center and determine whether there is a relationship between concentration of urine myoglobin(Mb) and acute renal failure(ARF) as a complication of MU. Serum and urine Mb were measured by RIA using myoglobin kit (Daiichi, Inc., Tokyo, Japan). The most common disorder of MU was septic shock with hypotension, followed by crush syndrome, major arterial occlusion by thormbosis, alcohol intoxication with status epilepticus, intoxication of unidentified snake venom and drug ingestion. On the basis of this limited amount of data, there is a significant association between high concentration in urine Mb(> 300 ng/ml) and ARF(Fisher's exact test, p< 0.005). To minimize the chances of development of ARF, routine urine Mb levels should be checked on patients at risk, especially septic shock with hypotension.

Acute renal failure secondary to myoglobinuria.

Acute Renal Failure (ARF) secondary to rhabdomyolysis and myoglobinuria was seen in four patients. In three, this was secondary to trauma and the fourth patient had an inflammatory myositis. All 4 patients had total recovery of renal function.

Sobrevida y mortalidad en pacientes con insuficiencia renal aguda: nuestra experiencia en el Hospital José María Cullen de Santa Fe / Survival and mortality of the patients with acute kidney failure: our experience to Hospital José María Cullen, Santa Fe

Se analizaron 39 pacientes consecutivos con IRA que requirieron hemodiálisis. Se observó su evolución teniendo en cuenta: situaciones clínicas, variables demográficas, severidad de la IRA, agresiones agudas, factores de riesgos predisponentes y complicaciones. Se observó una mortalidad global del 33,3%, una altísima incidencia de absortos sépticos (41%) con una mortalidad elevada en este subgrupo (37,5%) y una escasa incidencia de IRA de causas puramente obstétricas con mortalidad nula. Se observó una relativa benignidad de la IRA no oligúrica. Se encontró que el número de agresiones agudas influyen negativamente en la evolución, probablemente a través de un efecto aditivo en la severidad de la IRA. Se confirmó que la enfermedad renal preexistente es un importante factor de riesgo para el desarrollo de la IRA y que no tiene influencia negativa en el pronóstico. Se encontró una correlación positiva entre la mortalidad y el número de complicaciones. Se concluye que la mortalidad global no es un buen parámetro para valorar la atención de los pacientes con IRA; sí, en cambio, sería la mortalidad según subgrupos bien definidos en cuanto a la situación clínica