|Year : 2022 | Volume
| Issue : 1 | Page : 40-42
Diffuse alveolar hemorrhage secondary to immune thrombocytopenic purpura: a case report and literature review
Sheetal Chaurasia1, Ramya Raghavan2, Manjunath Thimmappa3
1 Department of Pulmonary Medicine, Manipal Hospital Whitefield, Bangalore, Karnataka, India
2 Department of Internal Medicine, Manipal Hospital Whitefield, Bangalore, Karnataka, India
3 Department of Critical Care Medicine, Manipal Hospital Whitefield, Bangalore, Karnataka, India
|Date of Submission||23-Jul-2021|
|Date of Acceptance||18-Oct-2021|
|Date of Web Publication||19-Apr-2022|
Dr. Sheetal Chaurasia
Department of Pulmonary Medicine, Manipal Hospital Whitefield, Bangalore, Karnataka, Postal Code: 560066
Source of Support: None, Conflict of Interest: None
Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening disease that can be caused due to several underlying disorders. The diagnosis and management of DAH remains challenging given its nonspecific presentation and lack of uniform criteria for the diagnosis. The management of DAH involves treating the causative underlying condition. On extensive review of the literature, only fewer than 10 cases of DAH associated with immune thrombocytopenic purpura (ITP) were found. The outcomes in DAH associated with ITP were also found to be variable in the reported cases, ranging from clinical recovery to mortality. We present a 45-year-old man with ITP who developed DAH, and a review of the available literature.
Keywords: DAH, DAH in ITP, diffuse alveolar hemorrhage, immune thrombocytopenic purpura, ITP
|How to cite this article:|
Chaurasia S, Raghavan R, Thimmappa M. Diffuse alveolar hemorrhage secondary to immune thrombocytopenic purpura: a case report and literature review. J Assoc Chest Physicians 2022;10:40-2
|How to cite this URL:|
Chaurasia S, Raghavan R, Thimmappa M. Diffuse alveolar hemorrhage secondary to immune thrombocytopenic purpura: a case report and literature review. J Assoc Chest Physicians [serial online] 2022 [cited 2022 May 28];10:40-2. Available from: https://www.jacpjournal.org/text.asp?2022/10/1/40/339688
| Introduction|| |
Immune thrombocytopenic purpura (ITP) is an autoimmune condition that is characterized by low platelet count and the presence of mucocutaneous bleeding. Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening condition that can be caused due to a number of underlying disorders. DAH refers to a subset of pulmonary hemorrhage that takes origin from the pulmonary microcirculation including alveolar arterioles, venules, and capillaries. DAH has been widely reported in association with various autoimmune conditions such as systemic lupus erythematosus, anti neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and antiphospholipid antibody syndrome but the association between DAH and ITP has been very scarcely reported in the literature with less than 10 reported cases., We present a 45-year-old man with ITP who developed DAH, and a review of the available literature.
| Case report|| |
A 45-year-old man presented with a cough, fever, hemoptysis, and sudden-onset dyspnea for 2 days. His medical history was significant for ITP, diagnosed 4 years ago for which he was treated with prednisolone and had been in remission for about a year. On clinical examination, he had a tachycardia of 116 beats per minute, tachypnea of 32 breaths per minute, and fever of 100°F with hypoxia SpO2 of 86%. Examination of his chest showed evidence of bilateral coarse inspiratory crepitations. He did not have any evidence of petechiae, ecchymosis, other bleeding manifestations, or splenomegaly.
On workup, he was found to have an elevated leukocyte count of 18,700/µL, normal hemoglobin levels of 14.7 g/dL, and a low platelet count of 41,000/µL. His prothrombin time-International normalized ratio and activated partial thromboplastin time were normal, as were his liver and renal function tests. His inflammatory markers were raised. An arterial blood gas analysis showed features of type 1 respiratory failure with hypoxemia and normocapnia. His chest X-ray on admission showed bilateral diffuse airspace consolidation. Blood and sputum cultures were negative.
He was admitted to the high dependency unit and started on broad-spectrum antibiotics and oxygen through face mask. His clinical conditions remained the same; however, his platelet count dropped to 19,000/µL and persisted to have hemoptysis. He received platelet transfusion and platelet count improved to 33,000/µL. A repeat chest X-ray showed worsening infiltrates. A computed tomography (CT) thorax was performed, which showed bilateral diffuse ground-glass opacities with patchy consolidation [Figure 1]. In view of persisting hemoptysis without an evident cause, he underwent a bronchoscopy.
|Figure 1 A computed tomography thorax showing bilateral diffuse ground-glass opacities with patchy consolidation.|
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Broncho alveolar lavage (BAL) with instillation of serial aliquots of 30 to 60 mL of normal saline showed a return of increasingly bloody lavage fluid [Figure 2]. BAL culture was sterile. Abundant hemosiderin-laden macrophages were identified in the BAL fluid was in favoring DAH.
|Figure 2 Bronchoalveolar lavage showing serial aliquots of increasingly bloody lavage fluid.|
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With a confirmed diagnosis of DAH, which was probably secondary to the underlying ITP, as evidenced by negative urinalysis and autoimmune and vasculitis profile, he was pulsed with intravenous methylprednisolone 1 g/day for 3 days followed by 500 mg/day. With the steroid pulse, his hemoptysis ceased, and his platelet count showed an improving trend to 120,000/µL. A repeat arterial blood gases showed resolution of his respiratory failure and a repeat chest X-ray showed improvement. He was discharged on oral prednisolone with a plan to taper and stop on follow-up.
| Discussion|| |
The DAH can be caused by numerous conditions, with most causes sharing a common pathophysiology of damage to the alveolar microcirculation. Any injury to the alveolar microcirculation can cause alveolar hemorrhage and these may include diffuse alveolar damage caused by infections, vasculitis of the alveolar blood vessels in conditions such as microscopic polyangiitis, immunologic damage to alveoli in conditions such as in Goodpasture syndrome and connective tissue disease, in coagulation disorders such as thrombotic thrombocytopenic purpura, precipitated by drugs such as anticoagulants. It can also found in association with other conditions such as idiopathic pulmonary hemosiderosis.
The ITP most commonly occurs between 18 and 40 years of age and is more common among women than men. ITP can produce platelet-type bleeding including petechiae, purpura, or other types of mucocutaneous bleeding including alveolar hemorrhage, albeit rarely. The goal for treatment of ITP is to maintain a safe platelet count to prevent bleeding. The decision to treat is based on platelet count, bleeding, and lifestyle of individual patients.,
Corticosteroids are the backbone of initial treatment. The treatment usually begins with IV methylprednisolone with or without immunoglobulin, along with platelet infusions to raise the count quickly in cases of major bleeding. Splenectomy is successful in about 60% of ITP cases. Steroid sparing immunosuppressives such as mycophenolate mofetil and azathioprine have shown varying degrees of effectiveness.
On an extensive review of the literature, we were able to identify fewer than 10 cases of DAH associated with ITP. The outcomes in DAH associated with ITP were also found to be variable in the reported cases, ranging from clinical recovery to mortality.,,
As DAH is rare, a high degree of clinical suspicion is required for early diagnosis. The usual triad of DAH may include hemoptysis, alveolar infiltrates on chest radiograph, and anemia; however, these are not specific for DAH. In our patient, he only had hemoptysis and alveolar infiltrates in the chest X-ray. His hemoglobin levels were well preserved, highlighting the fallacy of this triad. Physical examination is also usually nonspecific. The chest X-ray may be abnormal but shows nonspecific bilateral diffuse alveolar infiltrates, which may be better visualized on a CT. A definitive diagnosis is made by an early bronchoscopy in most patients who are suspected of suffering from DAH. Bronchoscopy may be useful for both documentation of the alveolar hemorrhage as well as the exclusion of infections, which are not easily distinguishable on radiologic imaging. In DAH, an increasing red blood cell (RBC) count in serial BAL aliquots from the same anatomical site is considered diagnostic. The histopathology in DAH includes intra-alveolar RBCs and fibrin, with the accumulation of hemosiderin-laden macrophages.,,In our patient, his clinical presentation was nonspecific, as were his laboratory and radiologic investigations. However, a relatively early bronchoscopy enabled us to establish a diagnosis in the early course of the disease and subsequently institute the management of the probable underlying cause; his ITP. Our patient responded to pulsed steroids and this reflected in the improvement of his underlying ITP with a serial rise in platelet count as well as cessation of the hemoptysis. Hence, early recognition of signs, diagnosis, and aggressive therapy of the underlying cause may likely decrease morbidity and mortality in DAH.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]