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PubMed1999

Microvesicular steatosis, hemosiderosis and rapid development of liver cirrhosis in a patient with Pearson's syndrome.

BACKGROUND/AIMS: Pearson's marrow-pancreas syndrome consists of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreas dysfunction. Patients with this disease usually have large deletions of the mitochondrial genome. We report a patient with Pearson's syndrome who had predominantly hepatic manifestations such as microvesicular steatosis, hemosiderosis and rapidly developing cirrhosis. METHODS: Analysis of the mitochondrial and nuclear genomes, determination of enzyme activities and of the hepatic iron content were performed using standard techniques of molecular biology and biochemistry. RESULTS: The patient had typical ringed sideroblasts in a bone marrow smear and a 7436-bp deletion of the mitochondrial genome in all tissues investigated, compatible with Pearson's syndrome. He died within 3 months after birth due to liver failure. Histopathological analysis of the liver revealed complete cirrhosis with signs of chronic cholestasis, microvesicular steatosis and massive hemosiderosis. In addition, the patient was heterozygous for the C282Y and H63D mutations of the hemochromatosis gene. CONCLUSIONS: Pearson's syndrome should be added to the list of neonatal diseases which can cause microvesicular steatosis, hepatic accumulation of iron and liver cirrhosis.

Disease Progression↗

PubMed2002

Prolonged liver dysfunction caused by hemosiderosis in a renal transplant recipient.

BACKGROUND: Liver dysfunction is a frequent complication that arises in the period following kidney transplantations, often resulting in death. We reported a case proving hemosiderosis as a cause of prolonged liver dysfunction after cadaveric kidney transplantation. METHODS: A 47-year-old man, who had been undergoing hemodialysis, was referred to our hospital on 2 November 1999. On the same day, cadaveric kidney transplantation was performed, and serum creatinine level reached a normal level within 2 weeks after surgery. However, serum transaminase gradually increased in the postoperative period. Serum ALT rose up to 116 IU/L on day 20 after the operation and 215 IU/L on day 30. Microscopic examination by needle biopsy revealed hemosiderosis of the liver. Recombinant human erythropoietin was administered and phlebotomy was performed. Liver function improved as a result. CONCLUSION: Early histological diagnosis can be a useful marker in predicting the course of chronic liver disease.

Cadaver↗

PubMedDate not supplied

[Idiopathic lung hemosiderosis in a 3-year-old child following ductus ligation in the neonatal period].

A premature girl (birth weight 1 405 g, gestational age 32 weeks) was mechanically ventilated for 36 days because of respiratory distress, pulmonary hemorrhage and ductus arteriosus. The hemodynamically active ductus was ligated on the 22nd day of life. At the age of three years a mycoplasma pneumonia and a pulmonary hemosiderosis developed. The possible etiologic role of the neonatal lung problems and the mycoplasma infection for the pulmonary hemosiderosis is discussed. Because a similar case has thus for not been reported the coincidence may be by chance.

Bronchopneumonia↗

PubMed1982

Hemosiderosis: an unrecognized complication in renal allograft recipients.

During the past decade, 258 patients received 293 renal transplants at Hennepin County Medical Center Minneapolis, Minnesota. Twenty-three of these patients had a pretransplant liver biopsy. Thirty-six patients had liver biopsies done during the post-transplant period. Liver tissue at autopsy was available from 24 patients who died after receiving a kidney transplant. Systematic analysis of the biopsy and autopsy specimens with appropriate tissue stains showed significant iron deposition (3+ to 4+) within the hepatic parenchymal cells in 1 of the 23 (4%) pretransplant biopsies and 17 of the 60 (28%) post-transplant specimens (P = 0.02). Eight of the 17 (47%) patients with post-transplant hemosiderosis also exhibited histological features of liver cirrhosis. As hemosiderosis is reversible with intermittent phlebotomies, early recognition and adequate management are highly desirable. We suggest that the serum ferritin level, which is a reliable index of body iron stores, should be monitored in all patients at periodic intervals following renal transplantation.

Adolescent↗

PubMed2005

Hemosiderosis is associated with accelerated decompensation and decreased survival in patients with cirrhosis.

AIM: Although hepatic iron deposition unrelated to hereditary hemochromatosis is commonly observed in cirrhosis, its clinical significance is unclear. The aim of this study was to examine the outcomes of cirrhotic patients with and without hemosiderosis. METHODS: Patients with an initial liver biopsy demonstrating cirrhosis between January 1993 and December 1997 were identified using the Department of Pathology database. Based on iron staining, patients were characterized as siderotic or nonsiderotic. Charts were reviewed to determine outcomes. RESULTS: Siderotic patients had significantly higher Child-Pugh (CP) and model for end-stage liver disease (MELD) scores. Their median survival without transplant was 23 months vs. 85 months in the nonsiderotics (P<0.0001, confidence interval: 95%). On univariate analysis, siderosis was associated with a hazard ratio of 2.74 (P<0.0001). On multivariate analysis, the effect of siderosis was reduced but remained significant after correction for the CP or MELD score (hazard ratios 1.82 and 2.06, P=0.05 and 0.02, respectively). Child's A cirrhotics with hemosiderosis decompensated more rapidly and had shorter median survival than those without siderosis (P=0.007 and P=0.01, respectively). CONCLUSIONS: The presence of siderosis is associated with more advanced liver dysfunction. Even when the effects of baseline liver function are taken into account, siderosis is associated with decreased survival and more rapid decompensation in cirrhosis.

Female↗

PubMed1998

Diffuse pulmonary hemosiderosis after exposure to pesticides. A case report.

This report describes the clinical, radiological, microscopical and ligandohistochemical findings in a 17-year-old woman who suffered from an acute onset of pulmonary hemosiderosis after inhalation of pesticides used for the cultivation of strawberries. She complained of headache, dyspnea, rhinitis, weakness and recurrent severe hemoptysis. Chest radiographs revealed bilateral patchy infiltrates, predominantly in the lower parts of both lungs. The consecutive severe anemia was treated by multiple blood transfusions which were repeated every 4-5 days. Open lung biopsies displayed signs of diffuse hemorrhage with hemosiderin-loaded macrophages, some hyaline membranes, focal fibroid deposits with intermingled histiocytes, mild interstitial fibrosis and focal intra-alveolar calcified bodies surrounded by foreign body giant cells. Analysis of endogenous lectins failed to demonstrate expression of binding capacities for maltose, fucose, mannose, lactose and sialic acid, Neither binding capacities for the macrophage-migration-inhibitory factor nor its presence, as analyzed by labeled sarcolectin, could be detected histochemically. The light microscopical findings are consistent with a longer-lasting diffuse pulmonary hemosiderosis; the presence of calcified bodies and foreign body giant cells (including the ligandohistochemical data) argues for a causal role of inhaled substances. The patient's clinical course improved after cyclophosphamide treatment, which restored her ability to work and released her from the need for recurrent blood transfusions.

Adolescent↗

PubMed1978

Hemosiderosis secondary to chronic parenteral iron therapy in maintenance hemodialysis patients.

Autopsy data on 24 chronic maintenance hemodialysis patients who had received varying doses of parenteral iron as the iron-dextran complex were reviewed for evidence of iron overload (hemosiderosis) and tissue fibrosis or organ dysfunction (hemochromatosis). Hemosiderosis was frequent in patients who received high total doses of iron but absent in those who received little or no iron. The degree of tissue iron did not increase with increased iron administration above a total of 2.5 g. Hemochromatosis or organ dysfunction secondary to tissue iron deposition was not noted in any patient. Chronic parenteral iron administration may improve anemia and result in tissue iron deposition but does not lead to hemochromatosis.

Adult↗

PubMed1991

Successful treatment of hemosiderosis with regular phlebotomy and recombinant human erythropoietin. Case report and ferrokinetic studies.

A 55-year-old female patient with hemosiderosis induced by administration of excessive doses of parenteral iron was successfully treated with regular phlebotomy combined with recombinant human erythropoietin (rHuEPO). Ferrokinetic data before therapy showed 28.0 mumol/l of serum iron, 4.1 mumol/l of unsaturated iron-binding capacity, 4,060 ng/ml of serum ferritin, 148 min of plasma iron disappearance time, 45% of red cell iron utilization and 0.4 mg/kg/day of plasma iron turnover rate. She had 300-ml phlebotomies, first every other week then weekly, and subcutaneous injections of rHuEPO twice a week. Two years later, the total volume of phlebotomized blood reached 31 liters and her ferrokinetic data showed: serum iron 8.6 mumol/l, iron-binding capacity 39.6 mumol/l, serum ferritin 277 ng/ml, plasma iron disappearance time 52 min, red cell iron utilization 100% and plasma iron turnover rate 0.5 mg/kg/day. During the phlebotomy therapy, her hemoglobin levels were maintained above 12 g/dl. No adverse effect due to rHuEPO occurred. These findings provide evidence for the efficacy of rHuEPO in multiple phlebotomy therapy for hemosiderosis and may open new avenues for its clinical application.

Bloodletting↗

PubMed1976

Idiopathic pulmonary hemosiderosis: evidence of capillary basement membrane abnormality.

Electron microscopic examination of a lung biopsy obtained from a child in the active stage of idiopathic pulmonary hemosiderosis disclosed prominent changes in the basement membrane of alveolar capillaries. Review of the pertinent literature showed that a structural defect of lung capillary walls in this disease has been proposed in the past mainly on theoretic grounds but not adequately documented at the ultrastructural level. Concentric multilayering of pulmonary capillary basal lamina, one of the changes observed in our material, is a rare phenomenon in this location, although one that has been well described in extrapulmonary microcirculatory beds and is probably a nonspecific reaction to vascular injury. The conceptual difficulties attending a definition of idiopathic pulmonary hemosiderosis are breifly discussed.

Basement Membrane↗

PubMed1986

Nitroglycerin-responsive pulmonary hypertension in idiopathic pulmonary hemosiderosis.

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon disease found predominantly in pediatric patients. It can produce severe chronic pulmonary injury that results in chronic hypoxemia, pulmonary insufficiency, and progressive pulmonary fibrosis, leading to irreversible pulmonary hypertension and death. We studied the pulmonary hemodynamics in an 9-yr-old boy with IPH to determine if pulmonary hypertension contributed to exacerbations of this disease. Our results showed that this patient demonstrated pulmonary hypertension during acute exacerbations. Initially, the elevated pulmonary artery pressure responded both to oxygen and to a pulmonary vasodilator in the form of nitroglycerin. However, this improvement was not sustained. We conclude that pulmonary hypertension is probably a result of chronic hypoxemia experienced by patients with pulmonary hemosiderosis. Further investigation is warranted to assess whether or not intervention aimed at reducing pulmonary artery pressure in IPH improves outcome.

Child↗

PubMed1977

Immunologic studies in cow's milk-induced pulmonary hemosiderosis.

Antibodies to cow's milk proteins (CMP) were studied by radioallergosorbent tests to determine the quantity and immunoglobulin class of these antibodies in nine patients with precipitins to cow's milk. Three of these patients had pulmonary hemosiderosis (PH); in one other patient pulmonary hemosiderosis was suspected, but not proven. Three patients had nonhemosiderosis chronic lung disease and two had other diseases. Age-matched control subjects were also studied. The quantity and immunoglobulin class distribution of antibodies to CMP were similar in all patients; the quantity of anti-CMP antibody was significantly greater in patients than in control subjects (P less than 0.001). In addition, complement-fixing antibody to CMP was not detected in either patients' or controls' sera. Lymphocyte responses to CMP were studied in three patients with PH, three individuals with other manifestations of milk hypersensitivity (positive controls), and nine negative control subjects. Patients and positive control subjects responded to CMP with greater tritiated thymidine incorporation than the negative control subjects (P less than 0.05). However, no significant difference in response to CMP was observed between the PH patients and the positive control subjects. Antibodies to human lung tissue were studied in the sera of all patients with PH and two negative control subjects. Antibodies to human lung and rat lung were detected in one patient with PH.

Antibodies↗

PubMed1992

Treatment of life-threatening primary pulmonary hemosiderosis with cyclophosphamide.

This report describes a five-year-old boy with severe pulmonary hemorrhage caused by primary pulmonary hemosiderosis with cow's milk sensitivity. After failing to respond to corticosteroids and azathioprine, he dramatically improved after being given cyclophosphamide. He worsened after discontinuation of cyclophosphamide on two occasions and improved significantly with its reinstitution. Cyclophosphamide was continued for 14 months without further bleeding or adverse effects. The patient has remained in remission for nearly five years. Cyclophosphamide may be a life-saving alternative therapy for refractory pulmonary hemorrhage with pulmonary hemosiderosis.

Child, Preschool↗

PubMed1993

Progressive massive fibrosis associated with idiopathic pulmonary hemosiderosis.

Progressive massive fibrosis is a recognized complication of pneumoconiosis secondary to pulmonary fibrosis, with retraction forming conglomerate masses symmetrically in the upper lobes. Idiopathic pulmonary hemosiderosis is known to cause pulmonary fibrosis secondary to recurrent alveolar hemorrhage with deposition of hemosiderin in the interstitium. We present a case in which progressive massive fibrosis developed in a patient with long-standing idiopathic pulmonary hemosiderosis.

Female↗

PubMed1997

Environmental risk factors associated with pediatric idiopathic pulmonary hemorrhage and hemosiderosis in a Cleveland community.

BACKGROUND: Unexplained pulmonary hemorrhage and hemosiderosis are rarely seen in infancy. A geographic cluster of 10 infants with this illness was identified in a large pediatric referral hospital in Cleveland, Ohio, during the period of January 1993 through December 1994. One infant died of severe respiratory failure. METHODS: A case-control study was conducted. Three control infants were matched by age with each case infant. All study infants' guardians were interviewed. Questions were asked about child care practices and home conditions for the period before case infants' illnesses. All infants' records were reviewed, their homes were visited, and a structural and environmental survey was conducted. RESULTS: All 10 case infants were black, and 9 were male, whereas 50.0% of control infants were male, and 83.3% were black. The case infants' mean age was 10.2 weeks (range, 6 weeks to 6 months). Matched analysis demonstrated that case infants' homes were more likely to have had water damage preceding the pulmonary hemorrhage event (odds ratio, 16.25; 95% confidence interval, 2.55 to infinity). Case infants were also more likely to have had close relatives with pulmonary hemorrhage (odds ratio, 33.14; 95% confidence interval, 5.10 to infinity). In addition, 50.0% of case infants experienced recurrent pulmonary hemorrhaging after returning to their homes. CONCLUSION: The results of this investigation of a cluster of infants with massive, acute pulmonary hemorrhage and hemosiderosis suggest that the affected infants may have been exposed to contaminants in their homes. Epidemiologic clues, such as water damage in the case infants' homes, suggest that environmental risk factors may contribute to pulmonary hemorrhage.

Acute Disease↗

PubMed2006

Hepatic adenomatosis associated with hormone replacement therapy and hemosiderosis: a case report.

We have reported a case of hepatic adenomatosis associated with hormone replacement therapy (estrogen and progesterone) and hemosiderosis caused by excessive blood transfusion for the treatment of chronic myeloid leukemia. A 34-year-old woman was found to have several hepatic tumors on a routine medical examination. The general condition was good. Laboratory studies showed iron overload. Abdominal computed tomography and selective hepatic angiography showed several hypervascular tumors in the right lobe of the liver (up to 20 mm in diameter). Since hepatocellular carcinoma could not be ruled out, subsegmental hepatectomy was performed. Histopathological examination of the surgical specimen showed hepatic adenomatosis with hemosiderosis. Both hormone replacement therapy and iron overload could be the cause of hepatic adenomatosis.

Adenoma↗

PubMed2001

Metastatic carcinomatous cirrhosis and hepatic hemosiderosis in a patient heterozygous for the H63D genotype.

A 38-year-old woman had a mastectomy for infiltrating ductal carcinoma of the breast 3 years before her last admission and had received chemotherapy for known liver metastases. She developed the rapid onset of liver failure with portal hypertension and died in a hospice. Autopsy revealed macronodular cirrhosis of the liver secondary to metastatic carcinoma of the breast with associated florid fibrosis. This rare lesion, previously called metastatic carcinomatous cirrhosis, was also found, in this case, to have marked hepatic hemosiderosis, and analysis of the patient's DNA showed heterozygosity for the H63D genotype. The possibility of cirrhosis-associated hemosiderosis secondary to an iron metabolism abnormality associated with the H63D mutation of the HFE gene is proposed. Computed tomographic scans showed the development of cirrhosis during the 3-month period before the patient's last admission and suggested the possibility of a postnecrotic type origin.

Adult↗

PubMedDate not supplied

Chloroquine in idiopathic pulmonary hemosiderosis. A case report.

This report describes an 11-year-old boy with idiopathic pulmonary hemosiderosis. His only presenting symptom was severe anemia due to iron deficiency. Idiopathic pulmonary hemosiderosis was diagnosed nine years after the onset of symptoms. During this period many invasive and non-contributory investigations were performed. This report describes the patient's diagnostic problems, clinical features and dramatic improvement with chloroquine (250 mg/day) after failing to respond to megadose methylprednisolone (30 mg/kg). One year later, chloroquine was discontinued. The patient has remained in remission since March 1994. Chloroquine should be used for this life-threatening condition since it is less toxic than other immunosuppressive drugs.

Anemia, Iron-Deficiency↗

PubMedDate not supplied

Pulmonary hemosiderosis with juvenile rheumatoid arthritis: a case report.

Pulmonary hemosiderosis may rarely be associated with juvenile rheumatoid arthritis or can develop during the course of the disease. We present a three-year-old boy with severe iron deficiency anemia (without any pulmonary symptoms) and arthralgia at the time of diagnosis. Two years after the initial diagnosis he developed pulmonary hemosiderosis and pauciarticular type of juvenile rheumatoid arthritis which progressed to seronegative polyarticular juvenile rheumatoid arthritis. He responded very well to prednisolone and was maintained well on low-dose alternate-day prednisolone and naproxen sodium treatment. This is the only case of association of these two diseases in our experience in both the Pediatric Rheumatology and Pediatric Respiratory Diseases Departments.

Anemia, Iron-Deficiency↗