Coagulopathy in GLA/GSD/KLA

Presented by Judith F. Margolin, MD, Texas Children’s Hospital, Houston, TX

Patients and their families face a veritable alphabet soup of acronyms starting with diagnoses (GLA/GSD/KLA), including diverse tests for diagnoses and monitoring (e.g., PT, PTT, Factors I-XII, d-dimers, DIC, LIC), and culminating with a host of procedures and drugs (generic and brand names)! Many of these terms, drugs, and procedures are related to coagulation.  This reflects the unfortunate complexity of lymphatic disorders.

Coagulation, also called thrombosis, it the process of forming platelet and protein plugs on torn or damaged blood vessels and tissues. It acts constantly as part of our body’s defenses, but it’s hard to get the right balance. Too little leaves bleeding and inflammation unaddressed; too much can impair circulation in the wrong place or, in the right place, but with disastrous effect.   Either type of problem contributes to pain and disability.  Lymphatic disorders often trigger both bleeding and clotting simultaneously.  Doctors and scientists have struggled for years to understand, predict, and alas, only partially control this complex sets of interactions. Fortunately, despite the limits of our knowledge, and the sometimes, unavoidable side effects of our therapies, it is possible to discern trends with the tests, intervene with drugs and procedures to “turn the tables” on the internal chaos of malfunctioning coagulation.

I hope to shed some light on the terms families and patients in the LGDA hear every day as they work with their care teams.  This brief talk can only skim the surface of a topic like this, but hopefully learning what is behind some of the jargon will clarify how we help patients with these disorders make progress towards living pain-free, normal lives.

About the Presenter

Dr. Margolin serves as Co-Director, Pediatric Hematology-Oncology Vascular Anomalies Program at Texas Children’s Hospital and is an Associate Professor, Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine in Houston, Texas. Her clinical interests include: leukemia, lymphoma, vascular anomalies, and hematologic disorders. She is board certified by the American Board of Pediatrics in general pediatrics and pediatric hematology-oncology.

Dr. Margolin received the LGDA Founder’s Award in 2018.

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