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Pulmonary alveolar septal calcinosis causing progressive respiratory failure in acute lymphoblastic leukemia in
Insights
Pulmonary alveolar septal calcification, a rare complication in children with acute lymphoblastic leukemia (ALL), can lead to fatal respiratory failure. Early diagnosis is crucial but challenging, as symptoms mimic other conditions.
Area of Science:
- Pediatric Oncology
- Pulmonary Medicine
- Pathology
Background:
- Acute lymphoblastic leukemia (ALL) is a common childhood cancer.
- Respiratory complications can arise in patients with ALL.
- Accurate diagnosis of pulmonary conditions in ALL patients is critical.
Observation:
- Two pediatric patients with ALL presented with a rare syndrome of pulmonary alveolar septal calcification, pneumothorax, and pneumomediastinum.
- Clinical and radiological findings initially suggested pulmonary edema or infection.
- Diagnosis was confirmed post-mortem, highlighting diagnostic challenges.
Findings:
- One patient had familial hyperparathyroidism; the other showed secondary hyperparathyroidism with osteitis fibrosa, suggesting hypercalcemia as a cause.
- Pulmonary calcinosis may result from hypercalcemia due to parathormone or prostaglandin-secreting tumors.
- Pneumothorax and pneumomediastinum might be caused by the rupture of calcified alveolar septa during mechanical ventilation.
Implications:
- This rare complication underscores the importance of considering metabolic derangements in ALL patients with respiratory distress.
- Further research is needed to determine if early diagnosis and intervention can improve outcomes.
- Understanding the pathogenesis of pulmonary calcinosis in ALL may lead to novel therapeutic strategies.
Abstract:
A syndrome of pulmonary alveolar septal calcinosis, pneumothorax, and pneumomediastinum, leading to rapidly progressive acute respiratory insufficiency and death was observed in 2 children with acute lymphoblastic leukemia (ALL). Primary clinical and radiological considerations in these patients were pulmonary edema and infection, and the diagnosis of pulmonary alveolar septal calcification was established only at autopsy. One patient, a 15-year-old girl, was found also to have parathyroid hyperplasia typical of familial hyperparathyroidism. The other, a 16-month-old girl, showed osteitis fibrosa of the bones and parathyroid hyperplasia of secondary type, suggesting that the pulmonary calcinosis resulted from hypercalcemia caused by a parathormone or prostaglandin-secreting tumor. The cause of pneumothorax and pneumomediastinum may have been rupture of calcified alveolar septa induced by high PEEP during ventilation of these patients. Other possible mechanisms contributing to hypercalcemia and pulmonary calcinosis in children with acute leukemia include bone resorption due to marrow infiltration, immobilization syndrome, renal failure, and administration of calcium, phosphate, or bicarbonate. This complication of acute leukemia in childhood is rare (2 patients in 430 autopsied over the period 1961-1982 at Childrens Hospital of Los Angeles). How often the process can be reversed if diagnosed before severe respiratory insufficiency is present is not known.
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