Case report |
https://doi.org/10.5005/ijrc-3-1-411 |
Multiple myeloma mimicking acute respiratory distress syndrome
Email: gokshe@gmail.com
Respiratory Therapy Intern
Professor of Anaesthesiology, Amrita Institute of Medical Sciences, Kochi
Abstract
Multiple myeloma involves lungs rarely but when it does, it may be difficult to differentiate it from other more common primary lung parenchymal, pleural diseases, tumours or metastatic diseases. This is a case report of a patient who developed clinical features of acute respiratory distress syndrome secondary to multiple myeloma with involvement of lung parenchyma by neoplastic plasma cells.
Keywords: Acute respiratory distress syndrome, multiple myeloma.
How to cite this article: Krishna G, Nair SG. Multiple myeloma mimicking acute respiratory distress syndrome. Ind J Resp Care 2014; 3(1): 411-4.
Introduction
Multiple myeloma is an incurable malignant plasma cell disorder that accounts for 20% of all deaths from haematologic malignancy and 2% of deaths from all cancers.1 Multiple myeloma is thought to arise from an asymptomatic premalignant stage of clonal plasma cell proliferation termed monoclonal gammopathy (MGUS).2 Pulmonary parenchymal involvement by multiple myeloma is very rare and the antemortem diagnosis of lung involvement by myeloma is difficult to distinguish as infection and alveolar haemorrhage have the same radiologic features.
Here, we report a rare case of multiple myeloma with pulmonary involvement which was mimicking acute respiratory distress syndrome (ARDS).
Case report
A 76 year old male who was admitted to our hospital complaining of generalised fatigue of six months duration and a slowly progressive, intermittent, nocturnal fever for the last 2 months. There was a history of significant weight loss for the last 6 months, decreased appetite and disorientation in the evening.
On physical examination, the patient appeared comfortable at rest but was febrile, tachypnoeic, tachycardic with a heart rate of 108/minute and a normal blood pressure. There were bilateral basilar crackles on auscultation and the oxygen saturation was 93% on 5 L oxygen through an oxygen mask. The chest radiograph showed bilateral multifocal areas of nodular consolidation (Figure 1). The electrocardiogram was normal. Routine blood evaluation showed that the patient was anaemic (haemoglobin 7.4 g %), with a very low total white cell counts (2.06 x 103 cells/cumm) with severe neutropaenia (< 500 cells/cumm). The platelet counts were within normal limits but the calcium levels were elevated (12.5 g/dL). Preoperative serology was negative. Peripheral blood smear showed normocytic, normochromic anaemia with leucopaenia and relative lymphocytosis. Initial arterial blood gas analysis showed severe lactic acidosis with a pH-7.27, PCO2-21 mm Hg, PO2-161 mm Hg, Lactate -11.8 mmol/L and serum HCO3− 12 mmol/L.
Figure 1: Chest x-ray showing bilateral multifocal areas of consolidation
The patient's respiratory status steadily declined over the ensuing days. Three days after admission, he was in acute respiratory distress with tachypnoea and the use of accessory muscles. Arterial blood gas showed moderate hypoxaemia with acidosis. The PaO2/FiO2 ratio was 250. He was intubated in view of impending respiratory failure and was mechanical ventilated with a protective lung strategy using low tidal volume (VT) and high positive end expiratory pressure (PEEP) protocol. A pressure regulated volume controlled (PRVC) mode of ventilation was used in the patient with a peak airway pressure (Ppeak) of 34 cmH2O.
A bronchoalveolar lavage revealed no growth or abnormal findings. Blood and urine cultures were negative. A computerised tomogram of the chest (CT scan) showed diffuse air space opacities with air bronchogram in both lung fields suggestive of pulmonary oedema. Multiple mediastinal lymph nodes were noted at paratracheal, para-aortic and subcarinal location, Patient was started on fluconazole empirically. He was also given beta blockers and intravenous frusemide considering a provisional diagnosis of pulmonary oedema. A bone marrow culture showed E.coli resistant to piperacillin-tazobactam and meropenem. Injection Amikacin was also started. A bone marrow aspiration showed evidence of infiltrative disease. A thoracoscopic biopsy was done which showed diffuse and nodular infiltration of lung parenchyma by neoplastic cells. A serum immune-electrophoresis showed hyperglobulinaemia with a monoclonal immunoglobulin G (IgG) kappa band and diagnosed as multiple myeloma with direct invasion of lung parenchyma.
Figure 2: CT scan showing diffuse air space opacities with air bronchogram in both lung fields
Discussion
This patient was diagnosed with multiple myeloma on the basis of a monoclonal serum protein IgG spike, hypercalcaemia, bone biopsy confirming infiltrative disease (lytic bone lesions) and infiltration of the lungs by neoplastic plasma cells. He developed clinical syndromes of ARDS secondary to this nodular involvement of the lungs by the myeloma. Infection as major contributing factor to the deteriorating lung condition was unlikely because the patient failed to respond to antibiotics radiographically or clinically, positive cultures from the lung were absent and positive pathological findings suggestive of malignant lung infiltration were present.
Multiple myeloma may involve the thorax in a variety of ways but pulmonary parenchymal involvement is uncommon. Thoracic manifestations include bony abnormalities, infiltration, pleural effusions, plasmacytomas and diaphragmatic dysfunction due to peripheral neuropathy. In a review of 958 patients with multiple myeloma at the Mayo Clinic, Kintzer et al reviewed the pleuropulmonary abnormalities in multiple myeloma.3 The abnormalities were chest roentgenograms. The most common finding was thoracic skeletal abnormality (28%), osteolytic lesions, localised or diffuse pulmonary infiltrates. Four patients had a diffuse infiltrate thought to be caused by a plasma cell infiltrate (proven in one). In 113 patients, plasmacytomas were seen (intramedullary 102, extramedullary 11). Pleural effusions were present in 58 patients (6%) and extramedullary plasmacytomas in 11 (1%).
In myeloma, respiratory failure from pulmonary involvement, although rarely reported, can include lung parenchymal involvement or the bony thoracic cage itself. Alveolar septal amyloidosis, plasma cell infiltration of lung parenchyma, accumulation of paraproteins, and metastatic calcification of the alveolar walls and blood vessels are all intra- parenchymal causes of respiratory failure from multiple myeloma.
Harinder et al reported a patient who had been diagnosed with multiple myeloma 5 years previously and presented with respiratory failure.4 He was found to have infiltration of lung tissue with plasma cells following radiotherapy for orbital plasmacytoma.
Chejfec et al reported two patients with bilateral lung densities on radiographic imaging without acute lung injury and also pathological evidence of neoplastic plasma cell infiltration of the lungs.5 He described these pulmonary infiltrates of neoplastic plasma cells with crystalline casts as ‘myeloma lung’.
Poe et al reported a patient with multiple myeloma and ARDS resulting from diffuse metastatic calcification of the alveoli and blood vessels.6 The lung calcification was probably due to renal failure induced hyperkalaemia. Respiratory insufficiency may occur in multiple myeloma even without parenchymal involvement. This may be due to bony abnormality leading to spontaneous flail chest and diaphragmatic involvement causing breathing dysfunction. Flail chest arises from multiple osteolytic rib lesions, leading to destabilisation of the rib cage, paradoxical breathing and respiratory failure.7,8 Diaphragmatic muscle weakness or paralysis results from chronic demyelinating polyneuropathy with phrenic nerve involvement.9
Marmor et al reported that parenchymal involvement with multiple myeloma should be included in the differential diagnosis of ARDS.10
Chest radiographs of direct involvement of neoplastic cells of myeloma may produce varied patterns including multiple masses imitating solid tumour metastasis, diffuse interstitial disease from alveolar septal amlyoidosis, or consolidation.11,12 Our patient presented with a chest radiographic pattern that was consistent with the diagnosis of ARDS, as defined by the American- European Consensus Conference on ARDS13 and ARDS Clinical Trials Network.14 Multiple myeloma with direct plasma cell invasion of pulmonary parenchyma can mimic or produce the clinical syndromes of ARDS and has its clinical implications.15
To conclude, it is important to consider pulmonary infiltration due to their primary disease in the differential diagnosis in patients known to have multiple myeloma as it can mimic ARDS.
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