Lymphatic abnormalities & inherited disorders -- e.g. Gorham's disease
FURTHER HISTORYThe diagnosis was established via an open lung biopsy.
are the PATHOLOGY SLIDES
CLICK on ANY SLIDE to see an ENLARGEMENT.
will notice the following features on the slides:
1) Marked vascular pulmonary thrombosis: arteries with focally marked medial and intimal hyperplasia consistent with hypertensive arteriopathy
2) Lymphangiectasia: Focally marked dilatation of pleural and interlobular septal lymphatic channels with edematous expansion of the interlobular septa
3) Pulmonary venous dilation: veins are dysmorphic
4) Patchy absence of airways: either bronchiolitis obliterans or small airway hypoplasia
5) Patchy acute inflammatory cell infiltrate within the interstitium
Virchow first described lymphangiectasia in 1856. It is the differential diagnosis for pulmonary cystic lesions, bilateral obstructive emphysema, and chronic asthma / pneumonia
Pathology: Pathologically, the lungs appear heavy and non-compliant. The visceral pleura have a network of dilated lymphatics that weep lymph when sectioned. Interlobular septa are widened and thickened. Additionally, lymphatic spaces are dilated and occasionally cystic. Small amount of collagen, smooth muscle may be found in vessel walls.
Three Diagnostic Classification Groups:
There are three diagnostic groups to consider within lymphangiectasia:
Primary Pulmonary Involvement
Secondary/ acquired PL from either Venous obstruction (eg intravascular thrombus) Cardiac morphological abnormalities
Generalized Lymphangiectasia: These patients usually have a less severe form of disease with mild G.I symptoms. Patient with generalized lymphangiectasia can have associated hypoproteinemia, edema and lymphopenia.