Hemosiderosis pulmonar idiopática (HPI): presentación de un caso y revisión de la literatura / Idiopathic pulmonary hemosiderosis (IPH): a case report and review of the literature

We present the case-report of a one-month-old infant, admitted to the Emergency Department with hypovolemic shock secondary to pulmonary hemorrhage who required life-support measures, including vasoactive drugs and methylprednisolone pulses. She was discharged from the hospital after 13 days of evolution and then readmitted 5 days later for a new episode of hemoptysis with hemodynamic compromise. Fiberoptic bronchoscopy was performed 4 days after the first episode showed a normal anatomy, without active bleeding, with 20% of hemosiderophages in bronchoalveolar lavage. Diffuse infiltrates were found on the chest radiograph. Differents studies were performed for check-out infection, heart disease, immune disease, thrombophilia, celiac disease, swallowing disorder, vascular abnormalities and allergy to cow's milk protein were negative, which led to Idiopathic Pulmonary Hemosiderosis (IPH). It was managed with amino acid formula, daily oral prednisone until 6 months of age and then every other day, and permanent inhaled fluticasone. In subsequent controls, normal growth and development were found, with no recurrences up to the time of this report, at 1 year of age. The favorable evolution in this case is attributed to early diagnosis and timely treatment with systemic corticosteroids. A review of the topic of IPH in pediatrics is presented, and study and treatment algorithms are proposed.

Se presenta el caso de una lactante de un mes de edad, que se presentó en el Servicio de Urgencia con shock hipovolémico secundario a hemorragia pulmonar. Necesitó medidas de soporte vital, incluyendo drogas vasoactivas y pulsos de metilprednisolona. Egresó del hospital a los 13 días de evolución y reingresó 5 días después por nuevo episodio de hemoptisis con compromiso hemodinámico. La fibrobroncoscopía efectuada a los 4 días de evolución del primer episodio mostró una anatomía normal, sin sangrado activo, con 20% de hemosiderófagos en el lavado broncoalveolar. En la radiografía de tórax se encontró infiltrados difusos. Los estudios en busca de infección, cardiopatía, enfermedad inmunológica, trombofilia, enfermedad celíaca, trastorno de deglución, anomalías vasculares y alergia a la proteína de la leche de vaca resultaron negativos, por lo que se planteó una Hemosiderosis Pulmonar Idiopática (HPI). Se manejó con fórmula aminoacídica, prednisona oral diaria hasta los 6 meses de edad y después en días alternos y fluticasona inhalada permanente. En controles posteriores se constató crecimiento y desarrollo normal, sin recidivas hasta el momento de este reporte, con 1 año de edad. La evolución favorable en este caso se atribuye al diagnóstico precoz y tratamiento oportuno con corticoides sistémicos. Se presenta una revisión del tema de HPI en pediatría y se proponen algoritmos de estudio y tratamiento.

Severe iron-deficiency anemia as initial manifestation of pulmonary hemosiderosis in a child / Anemia ferropriva grave como manifestaçao inicial de hemossiderose pulmonar em criança

ABSTRACT Idiopathic pulmonary hemosiderosis is a potentially fatal disease that results from episodes of alveolar hemorrhage of unknown origin. The clinical spectrum is varied, and anemia may constitute the only manifestation of illness, preceding other signs and symptoms by several months. We present the case of a 4 year-old child presenting with fever, vomiting and prostration, associated with pallor. He had microcytic and hypochromic anemia refractory to iron therapy. Gastrointestinal bleeding was ruled out after negative extensive etiological investigation. Subsequently, pulmonary infiltrates suggestive of alveolar hemorrhage were observed in the chest radiography. The cytological exam of the bronchoalveolar lavage showed hemosiderin-laden macrophages. After the etiological study, the diagnosis of idiopathic pulmonary hemosiderosis was made by exclusion. He was initiated on corticosteroid therapy, later associated to an immunosuppressive agent, with subsequent correction of anemia and of the radiological pattern. The patient is currently asymptomatic.

RESUMO A hemossiderose pulmonar idiopática é uma doença potencialmente fatal que cursa com episódios de hemorragia alveolar de etiologia desconhecida. As manifestações clínicas são variadas, e a anemia pode constituir o único sinal de doença, precedendo em vários meses os outros sinais e sintomas. Apresenta-se o caso de criança de 4 anos, com febre, vômitos e prostração, associados à palidez. Apresentava anemia microcítica e hipocrômica, refratária à terapêutica com ferro. A hipótese diagnóstica de sangramento gastrintestinal foi excluída, após investigação etiológica extensa, inconclusiva. Posteriormente, em radiografia torácica, foram observados infiltrados sugestivos de hemorragia alveolar. O exame citológico do lavado broncoalveolar mostrou macrófagos com depósitos de hemossiderina. Após estudo etiológico, assumiu-se, por exclusão, o diagnóstico de hemossiderose pulmonar idiopática. Foi iniciada terapêutica com corticoides, associada posteriormente a imunossupressor, com correção subsequente da anemia e do padrão radiológico, encontrando-se, atualmente, assintomático.

Hemosiderosis pulmonar idiopática con osificación pulmonar / Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification

La osificación pulmonar es un hallazgo poco usual, generalmente asintomático, que se reporta como incidental en biopsias de pulmón. Asimismo, la hemosiderosis pulmonar idiopática es una causa poco frecuente de infiltración pulmonar. Se presenta el caso de un hombre de 64 años con síntomas respiratorios crónicos, en quien se detectaron estas dos condiciones en el estudio histopatológico.

Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged

Hepatocarcinoma, porfiria y hemosiderosis. Una asociación no reportada en nuestro país: caso clínico / Porphyria cutanea tarda, hemosiderosis and hepatocellular carcinoma: report of one case

Porphyria cutanea tarda (PCT) is a hereditary or acquired disease. It can be unleashed by iron overload, alcohol, estrogens and other conditions. In these patients, hepatic involvement can be associated to cirrhosis, iron overload or C and B viral infections, that are predisposing factors for hepatocellular carcinoma. We report a 69-year-old man with PTC, hemosiderosis and hepatocarcinoma. The tumor was diagnosed during a routine ultrasound examination for early detection of malignant lesions. The patient was subjected to a right hepatic excision. The pathological examination of the surgical piece confrmed the diagnosis and disclosed free surgical margins. After 18 months of follow up, the patient had a relapse and a liver transplantation was performed.

Hemosiderose pulmonar idiopática: exacerbação associada à gestação / Idiopathic pulmonary haemosiderosis: exacerbation associated to pregnancy

A hemosiderose pulmonar idiopática (HPI) é uma desor­dem rara, caracterizada por episódios repetidos de hemorragia alveolar. A maioria dos estudos relatam casos de HPI na infância ou adolescência. Este trabalho relata um caso de hemoside­rase pulmonar idiopática com exacerbação durante a gestação. O manejo na gestação é complicado e há poucos casos descri­tos na literatura.

Associação de bronquiolite obliterante pós-infecciosa e hemossiderose pulmonar na infância / Postinfectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in childhood

Descreve-se uma apresentação rara de bronquiolite obliterante associada a hemossiderose pulmonar, em paciente de nove anos com sintomas respiratórios persistentes iniciados após episódio de bronquiolite aguda grave aos sete meses. Após o episódio agudo, apresentou sintomas respiratórios persistentes, piorando significativamente aos sete anos, quando começou a apresentar dificuldade respiratória em pequenos esforços. A tomografia computadorizada de tórax demonstrou achados compatíveis com bronquiolite obliterante. A biópsia pulmonar a céu aberto demonstrou numerosos macrófagos corados com hemossiderina, além dos achados compatíveis com bronquiolite obliterante. O diagnóstico de hemossiderose pulmonar pode estar ocasionalmente associado a bronquiolite obliterante em crianças com seqüela pós-viral grave.

In the present report, we describe an unusual presentation of post-infectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in a nine-year-old boy with persistent respiratory symptoms subsequent to an episode of acute bronchiolitis occurring at the age of seven months. After the episode, the persistent respiratory symptoms worsened significantly, and, by the age of seven, the patient began to have difficulty breathing after minimal exertion. Computed tomography of the chest presented findings consistent with bronchiolitis obliterans. Open lung biopsy revealed numerous hemosiderin-laden macrophages, as well as other findings consistent with bronchiolitis obliterans. Pulmonary hemosiderosis can occasionally be accompanied by bronchiolitis obliterans in children with severe sequelae after an episode of viral infection.

Idiopathic pulmonary haemosiderosis: two case reports.

Idiopathic pulmonary haemosiderosis (IPH) is a rare disorder and is characterised by the occurrence of episodes of diffuse alveolar haemorrhage (DAH). The patients usually present with a history of recurrent haemoptysis, anaemia and alveolar opacities on chest radiograph. The radiographic features are very characteristic and reveal alveolar opacities in middle and lower zones with selective sparing of the upper zones. An extensive diagnostic work-up is needed to exclude other causes of DAH. We report two cases of idiopathic pulmonary haemosiderosis who responded well to treatment with oral glucocorticoids.

Mitral valve prolapse with pulmonary haemosiderosis and severe anaemia: cause or association?

A 12 years boy presented with the history of pallor for one month. Two days before hospitalisation he developed fever, cough, shortness of breath. He had past history of such episode.On examination, his heart rate was found to be 120/minute, respiratory rate 40/minute and moderate anaemia was detected. Scattered creptus was audible over mid and lower lung fields and a soft systolic murmur was auscultated at apex. On investigations, Hb was found as 4.6 g/dl and HbF was less than 2%. Plasma Hb was 5 g/dl. Straight x-ray chest showed bilateral patchy opacities over mid and lower zones. His sputum was found to be blood stained and prompted the possibility of blood loss occurring in the lungs, which was confirmed by demonstrating haemosiderin laden macrophages on three consecutive sputum specimens. Echocardiography revealed a systolic displacement of mitral valve leaflets into the left atrium with co-optation superior to the plane of mitral annulus. Doppler study showed a minimal late systolic regurgitation. It was decided to treat the case as idiopathic pulmonary haemosiderosis. Oxygen inhalation, hypertonic saline nebulisation, i.v. hydrocortisone, packed cell transfusion followed by oral prednisolone improved the patient's condition. After 3 months of discontinuing prednisolone, he remained asymptomatic. Here one case of pulmonary haemosiderosis characterised by abnormal accumulation of haemosiderin in the lungs following repeated alveolar haemorrhages with the presence of mitral valve prolapse is reported.

Idiopathic pulmonary haemosiderosis: an unusual cause of recurrent haemoptysis.

Haemoptysis is a clinical problem which occurs in association with a variety of disorders. We report a patient who presented with the triad of haemoptysis, anaemia and diffuse alveolar consolidation suggesting diffuse pulmonary haemorrhage (DPH).

Hemossiderose pulmonar primaria na crianca: apresentacao de dois casos / Primary pulmonary hemosiderosis in children: presentation of two cases

Os autores apresentaram dois casos de hemossiderose pulmonar primaria em criancas, possivelmente relacionada a alergia ao leite de vaca. As manifestacoes clinicas caracterizam-se por tosse, dispneia, insuficiencia cardiaca congestiva associadas a pneumopatia de repeticao e anemia ferropriva. O diagnostico foi confirmado pelo encontro de hemossiderina em macrofagos do escarro no caso 1 e em macrofagos pulmonares no caso 2. Em ambos os casos, o quadro clinico sugeriu a relacao da doenca com alergia a leite de vaca (variante de Heiner).

Hemossiderose pulmonar idiopática do adulto: descriçäo de um caso / Adult idiopathic pulmonary hemosiderosis: report of a case

Os autores descrevem um caso de hemossiderose pulmonar idiopática do adulto em um paciente de 20 anos. Anemia ferropriva, hemoptises de repetiçäo e infiltrado pulmonar na radiografia de tórax säo as manifestaçöes encontradas nesse paciente. O diagnóstico foi confirmado com a biópsia transbrônquica, com achado de hemorragia alveolar e intersticial com macrófagos carregados de hemossiderina. O paciente foi tratado inicialmente com prednisona, mas foi necessária a associaçäo de ciclofosfamida, com remissäo clínica até o momento

Hemosiderosis pulmonar asociada con fibrosis pancreática: informe de un paciente / Pulmonary hemosiderosis associated with pancreatic fibrosis: report of a patient

Se presenta un caso de hemosiderosis pulmonar asociada con fibrosis pancreática en un niño de 17 meses de edad, cuyo diagnóstico fue establecido por la necropsia. Se discute la posibilidad de una base inmunológica del proceso. Se plantea la existencia de un nuevo síndrome: hemosiderosis pulmonar-fibrosis pancreática

Hemosiderosis pulmonar idiopática / Idiopathic pulmonary hemosiderosis

Se presenta la revisión retrospectiva de 28 casos de hemosiderosis pulmonar idiopática (HPI) vistos en el Hospital Infantil de México Federico Gómez. Los casos presentaron una distribución bifásica en cuanto a edad y sexo predominando en varones menores de un año y en mujercitas en edad escolar. La HPI se presentó como una neumopatía subaguda originada por sangrado en el parénquima pulmonar de severidad variable que evolucionó por meses años acompañada de palidez y fiebre y que condujo en la mayoría de los casos a insuficiencia cardiorespiratoria severa. La mayor parte de los casos (71%) presentó anemia regenerativa. De éstos, la mitad presentó también hipo o anisocromia. En tres de seis pacientes con anemia se observó patrón de deficiencia de hierro y en tres patrón de exceso. Estos últimos tendieron a ser aquellos con una evolución más prolongada. En ningún caso se pudo documentar una etiopatogenia autoinmune. La radiografía de tórax fue anormal en todos los casos. Las alteraciones predominantes fueron infiltrados micronodulares difusos, infiltrados algodonosos e imágenes de neumonitis En casos avanzados se observó fibrosis intersticial. En la mitad de los casos las imágenes pulmonares cambiaron en lapsos cortos de tiempo. Los casos han evolucionado en forma larvada con exacerbaciones periódicas y respuestas variable a esteroides. De los 128 casos, 17 han fallecido, de ocho se ignora su situación actual y tres continuaban vivos cinco a 37 meses después de iniciado su padecimiento. Se discute la posibilidad de diversos mecanismos etiopatogénicos en lo que tal vez sea el "síndrome" de HPI. Se discute la fisiopatología de la anemia y se enfatiza la necesidad de estudios inmunológicos tempranamente en la evolución de la enfermedad tratando de definir mecanismos etiopatogénicos que son esenciales para el mejor manejo del padecimiento

Hemosiderosis en pacientes portadores de linfomas, sin tratamiento previo: comunicación previa / Hemosiderosis in patients with lymphoma, without previous treatment

Ante lo categórico de los resultados de este estudio y apoyándose en publicaciones que describen alteraciones del metabolismo de hierro causalmente relacionadas con la inmunodeficiencia, es que decidimos iniciar un estudio cooperativo con la Sección Oncohematología con el fin de comprobar las siguientes hipótesis en pacientes portadores de linfomas: 1) Alteración en la absorción de hierro. 2) Alteración en el transporte de hierro. 3) Alteración en la captación de hierro por el hepatocito. 4) Correlación de la presencia de hierro en el hígado con la presencia de depósitos de hierro en médula ósea, con métodos de microscopía óptica y microscopía electrónica