2.2 Potential Complications
Patients with CLA suffer from pain and functional impairment,
psychosocial stress and decreased quality of life (QOL). CLA have
distinctive, yet often overlapping complications as below.
- Lytic bone lesions. Bone lesions may cause pain and predispose
to pathologic fractures. Pathologic fractures tend to occur most
frequently in GSD because of contiguous bone involvement and cortical
bone destruction. Lytic bone lesions usually do not occur in
individuals with CCLA.
- Coagulopathy. Patients with KLA develop severe thrombocytopenia
and hypofibrinogenemia, frequently causing bleeding complications.
GSD, GLA, and CCLA are not associated with coagulopathy.
- Chylous effusions. Accumulation of chylous pleural and
pericardial effusions, along with lymphatic ascites, may be severe and
lead to cardiopulmonary compromise or death. Chylous effusions may
occur in patients with all 4 CLA. However, effusions are usually
hemorrhagic in individuals with KLA.
- Protein losses. Loss of proteins and lymphocytes, sometimes in
massive quantities, may occur when lymphatic fluid accumulates in
effusions, particularly when surgically drained. Hypogammaglobulinemia
and lymphopenia may cause immunocompromise. Hypoalbuminemia leads to
decreased oncotic pressure resulting in generalized edema while other
protein losses may impair growth. Protein losses may occur in all 4
CLA but occurs most frequently with CCLA.