Thursday, March 26, 2015

Bat Man

A 40 year old man presents with insidious gradual shortness of breath. He gets dyspneic on exertion and has a cough, fatigue, weight loss, and low-grade fever. His physical exam is pretty much normal. His labs show polycythemia, hypergammaglobulinemia, and increased LDH. Chest X-ray is below:


High resolution CT scan shows homogeneous ground glass opacities. Pulmonary function tests show a restrictive ventilatory defect. A bronchioalveolar lavage results in an opaque milky fluid which settles upon standing.

Challenge: Aha! Your diagnosis is...?

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2 comments:

RaH said...

Polycythemia and Hypergammaglobulinamia are signs of lymphocytic and/or myelocytic proliferation. the chest ray shows sings of bilateral masses, peri-hiliar and basal shadowing.
it could be one of the Terrible T's: the terrible Lymphoma, eventhough usually only one of the cell lines is impaired (either Myeloic, or lymphoic cells...)
it could be a Thymoma, but this would usually cause a redcellaplaisa as well as myasthenia gravis..
i quote you from an erlier case:
Thymomas are associated with a paraneoplastic syndrome causing myasthenia gravis (present in 35% patients at diagnosis). (They are also associated with red cell aplasia and hypergammaglobulinemia). Myasthenia gravis is characterized by autoantibodies against the acetylcholine receptor. This causes the presentation described here. The pathogenesis is unknown.

but thats still just an educated guess.

Craig Chen said...

great reasoning - that's definitely on the differential
-
Bat Man

This is pulmonary alveolar proteinosis, a diffuse lung disease caused by accumulation of lipoproteinacious material (surfactant) in the distal air spaces. The chest radiograph shows bilateral symmetric alveolar opacities in the mid and lower lung zones, sometimes in a “bat wing” appearance.

Source: UpToDate.