scholarly journals Cobalamin C deficiency presenting with diffuse alveolar hemorrhage and pulmonary microangiopathy: a case series of four patients

2019 ◽  
Author(s):  
Jinrong Liu ◽  
Xiaolei Tang ◽  
Chunju Zhou ◽  
Hui Xu ◽  
Haiming Yang ◽  
...  
Keyword(s):  
Lung Disease ◽  
Late Onset ◽  
Clinical Manifestations ◽  
Case Series ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Cobalamin Deficiency ◽  
Ground Glass ◽  
High Dose ◽  
Compound Heterozygous

Abstract Background: The clinical manifestations of combined methylmalonic acidemia (MMA) and homocysteinemia (cobalamin deficiency) vary, but typically include neurologic, developmental and hematologic abnormalities. Only a few patients had diffuse lung disease (DLD). We analyse the clinical features of cobalamin C deficiency (CblC deficiency) who developed late-onset DLD mainly diffuse alveolar hemorrhage (DAH) to strengthen an understanding of it.Results: This study describes 4 patients aged 4-years-2-months to 7-years-6-months with CblC deficiency who developed late-onset DLD. Of these, the first 3 patients presented predominantly with DAH, and the last patient with pulmonary microangiopathy confirmed by lung biopsy. All patients accompanied by pulmonary arterial hypertension (PAH), and 2 accompanied by moderate megaloblastic anemia. Diffuse ground-glass opacification and poorly defined ground-glass centrilobular nodules were seen on high-resolution computed tomography (HRCT) in 1 patient and 3 patients respectively. All patients were suspected of having idiopathic pulmonary hemosiderosis (IPH) or interstitial lung disease (ILD) at other hospitals. The last 2 patients received treatment with high dose corticosteroid before admission, but the symptoms didn’t improve. Moreover, all patients carried compound heterozygous mutations (c.80A>G, c.609G>A) and improved significantly after being treated for CblC deficiency and PAH.Conclusions: CblC deficiency should be considered in the differential diagnosis of DAH especially with PAH, and pulmonary microangiopathy be the main reason of DLD in these patients.

scholarly journals Severe Diffuse Alveolar Hemorrhage Associated with Bortezomib Administration in Patients with Multiple Myeloma

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5761-5761 ◽  
Author(s):  
Ayed O Ayed ◽  
Jan S Moreb ◽  
Jack W. Hsu ◽  
John W Hiemenz ◽  
John R Wingard ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Respiratory Failure ◽  
Respiratory Symptoms ◽  
Conflicts Of Interest ◽  
Early Recognition ◽  
Gastrointestinal Symptoms ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Ground Glass ◽  
High Dose

Abstract Background: Bortezomib, a proteasome inhibitor, is frequently used in treatment of patients with multiple myeloma (MM). Bortezomib is generally well tolerated with gastrointestinal symptoms and peripheral neuropathy being the most common adverse effects. Here we report cases of severe diffuse alveolar hemorrhage (DAH) associated with bortezomib administration. Methods and Results: We identified 3 cases of severe DAH that were associated with bortezomib administration in MM patients treated at our institution between 2010 and 2014 (Table 1). All 3 patients presented with fever and worsening hypoxia shortly after initiation of bortezomib therapy and later developed progressive respiratory failure due to DAH. None of 3 patients had any respiratory symptoms or preexisting pulmonary conditions prior to bortezomib initiation. In one patient respiratory symptoms developed after 4 doses of bortezomib, improved off therapy, however rapidly progressed to a respiratory failure with a subsequent bortezomib treatment. Imaging studies showed diffuse areas of ground-glass opacities, apparent prominence of segmental and subsegmental bronchi and interlobular septal thickening. Infectious workup, which included bronchoscopy with bronchoalveolar lavage and respiratory viral studies, was completely negative in all 3 patients. All patients received supportive care, empiric broad-spectrum antimicrobial therapy and high-dose glucocorticoids, which were started within 4 days of hospital admission. Two patients died from complications of DAH and 1 patient recovered without pulmonary sequelae. Conclusion: Bortezomib-associated DAH is a rare, but serious and potentially fatal adverse event. Pathogenesis remains unclear and is likely related to impaired regulation of the inflammatory response. Early recognition of bortezomib associated DAH is essential for immediate discontinuation of the drug and prompt initiation of high dose steroids. More reports are needed to provide further insights about the pathogenesis and most optimal management of bortezomib induced DAH. Abstract 5761. Table 1: Patients who developed DAH after bortezomib administration Pt Age/ Gender/Race Smoker Sx Bor dose Bor schedule # of Bor doses Time of onset after last bor dose (days) Platelet count at the onset (103/mm3) Imaging/BAL findings Time to initiation of high dose steroids Outcome 1 67/M/C Never SOB, fever 1.3 mg/m2 IV/2Xwk 8 5 157 Bilateral infiltrates and GGO/DAH 2 Death 2 72/M/C Yes Fever, cough 1.3 mg/m2 IV/2Xwk 2 3 108 Bilateral infiltrates/DAH 4 Recovery 3 55/M/C Yes Fever, chills 1.5 mg/m2 IV/2Xwk 1 2 144 Bilateral diffuse airspace disease/DAH 4 Death Abbreviations: Pt, patient; M, male; C, Caucasian; Sx, symptoms; Bor, bortezomib; BAL, broncho-alveolar lavage; GGO, ground-glass opacities. Disclosures No relevant conflicts of interest to declare.

scholarly journals Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis

2021 ◽  
Vol 84 (4) ◽  
pp. 255-262
Author(s):  
Min Jung Kim ◽  
Kichul Shin
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Microscopic Polyangiitis ◽  
Usual Interstitial Pneumonia ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Pulmonary Manifestations

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)‒associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

scholarly journals Catastrophic antiphospholipid syndrome presented with coronary thrombosis, renal impairment, and suspected diffuse alveolar hemorrhage treated with rituximab biosimilar (CT-P10)

2022 ◽  
Vol 20 ◽  
pp. 205873922110508
Author(s):  
Changgon Kim ◽  
Hyun-Sook Kim
Keyword(s):  
Cardiac Arrest ◽  
Renal Impairment ◽  
Antiphospholipid Syndrome ◽  
Coronary Thrombosis ◽  
Anticardiolipin Antibody ◽  
Coronary Intervention ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
High Dose ◽  
Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a lethal disease that occurs suddenly and progresses to multi-organ failure. We present a case of CAPS successfully treated with the rituximab biosimilar CT-P10. A 38-year-old man was referred with a sustained fever and unexplained elevated creatinine levels. Cardiac arrest by ventricular fibrillation occurred upon arrival at the hospital. We diagnosed probable CAPS because of coronary thrombus, renal impairment, suspected diffuse alveolar hemorrhage, and positive anticardiolipin antibody immunoglobulin G. We performed percutaneous coronary intervention for the cardiac arrest, and treated him with extracorporeal membrane oxygenation, mechanical ventilation, and continuous renal replacement therapy. When CAPS was diagnosed, we administered CT-P10 after administering high-dose glucocorticoid. Our case suggests that the use of a rituximab biosimilar is economically efficient in the treatment of CAPS, as in other rheumatic diseases. The patient was cured without recurrence at the 2-year follow-up.

Diffuse alveolar hemorrhage following intravenous thrombolytic treatment in acute ischemic stroke: a case series

2019 ◽  
Vol 41 (1) ◽  
pp. 233-237
Author(s):  
Murat Mert Atmaca ◽  
Ugur Burak Simsek ◽  
Ipek Midi ◽  
Rustem Aliev ◽  
Emrah Aytac ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Case Series ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Thrombolytic Treatment

Comparison of the Spectrum of Radiologic and Clinical Manifestations of Pulmonary Disease Caused by Mycobacterium avium Complex and Mycobacterium xenopi

2014 ◽  
Vol 65 (3) ◽  
pp. 207-213 ◽  
Author(s):  
Maria C. Carrillo ◽  
Demetris Patsios ◽  
Ute Wagnetz ◽  
Frances Jamieson ◽  
Theodore K. Marras
Keyword(s):  
Computed Tomography ◽  
Lung Disease ◽  
Pulmonary Disease ◽  
Clinical Data ◽  
Mycobacterium Avium ◽  
Mycobacterium Avium Complex ◽  
Clinical Manifestations ◽  
Ground Glass ◽  
Mycobacterium Xenopi ◽  
Significant Difference

Aim Mycobacterium xenopi is described with upper lobe cavitation (“fibrocavitary” pattern), whereas the Mycobacterium avium complex (MAC) is described with bronchiectasis and centrilobular nodules (“nodular bronchiectasis”). We retrospectively described and compared computed tomography (CT) chest manifestations of disease caused by MAC and M xenopi. Materials and Methods We reviewed patients who had either MAC or M xenopi lung disease and who had CTs between January 2002 and December 2003. Clinical data were recorded, and the patterns on chest CTs were categorized as “fibrocavitary,” “nodular bronchiectatic,” and “unclassified.” Results There were 74 patients; 50 with MAC and 24 with M xenopi. The patients with MAC were older (mean 69 vs 58 years; P = .007). Patients with M xenopi more often had emphysema (50% vs 20%; P = .02), cavities (46% vs 16%; P = .01), and nodules ≤5 mm (88% vs 58%; P = .02). M xenopi cases more commonly had a fibrocavitary radiologic pattern (33% vs 18%), with no statistically significant difference ( P = .24). MA C was more often associated with a nodular bronchiectatic pattern (68% MAC vs 4% M xenopi; P < .0001). Sixty-three percent of patients with M xenopi had a pattern that was predominantly randomly distributed nodules (11/15 [73%]) or consolidation and/or ground-glass opacities (4/15 [27%]). Conclusion Compared with MAC, patients with M xenopi infection develop more cavities and more nodules, and they less often have a predominant nodular bronchiectatic pattern. Although a predominantly cavitary pattern appears to be more common with M xenopi, the majority of patients with M xenopi had CT patterns of random nodules or consolidation and/or ground-glass opacities rather than classically described findings.

scholarly journals Diffuse Alveolar Haemorrhage: A Single Centre Experience

2019 ◽  
Vol 2 (2) ◽  
pp. 229-233
Author(s):  
Ashesh Dhungana ◽  
Prajowl Shrestha
Keyword(s):  
Respiratory Failure ◽  
Bronchoalveolar Lavage ◽  
Plasma Exchange ◽  
Renal Involvement ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Alveolar Haemorrhage ◽  
Remission Induction ◽  
High Dose ◽  
Systemic Corticosteroids

Introduction: Diffuse alveolar hemorrhage results from an accumulation of red blood cells into the alveolar space. Symptoms of alveolar hemorrhage are dyspnea, hemoptysis, anemia, diffuse pulmonary infiltrates and hypoxemic respiratory failure. Diagnosis is established by bronchoalveolar lavage and treatment includes a combination of high dose systemic corticosteroids, immunosuppressant and plasma exchange. The aim of this study is to evaluate the clinical radiological profile and laboratory findings and utility of bronchoalveolar lavage in the diagnosis of diffuse alveolar hemorrhage.Materials and Methods: In a retrospective review between February 2017 and December 2017, medical records of patients with a diagnosis of diffuse alveolar hemorrhage presenting at the National Academy of Medical Sciences, Kathmandu, Nepal, were analyzed. Clinical, radiology and laboratory results along with bronchoalveolar lavage results were extracted. Treatment received and clinical responses were evaluated.Results: A total of five patients were diagnosed to have diffuse alveolar hemorrhage based on bronchoalveolar lavage analysis. Three had hemorrhage secondary to Antineutrophil Cytoplasmic Antibody associated vasculitis, one had Systemic Lupus Erythematosus and the other Idiopathic Pulmonary Hemosiderosis. Renal involvement was present in three patients. All patients received systemic corticosteroids, three received Cyclophosphamide and one Rituximab for remission induction. Plasma exchange was done in two patients with severe hypoxemia. Of the five patients, four improved whereas one died.Conclusions: Diffuse alveolar hemorrhage presents with non-specific symptoms. Bronchoalveolar lavage is extremely useful to establish the diagnosis and exclude infections. Early initiation of immunosuppressant prevents respiratory failure and death.

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