How is Kasabach-Merritt syndrome diagnosed?
See how Kasabach-Merritt syndrome is diagnosed. Which specialists are essential to meet, what tests are needed and other useful information for the diagnosis of Kasabach-Merritt syndrome
Kasabach-Merritt syndrome (KMS) is a rare condition characterized by the presence of a vascular tumor, typically a hemangioma, along with severe thrombocytopenia (low platelet count) and consumptive coagulopathy (abnormal blood clotting). Diagnosing KMS involves a combination of clinical evaluation, laboratory tests, and imaging studies.
Clinical evaluation: The first step in diagnosing KMS is a thorough physical examination by a healthcare professional. They will assess the patient's medical history, symptoms, and perform a comprehensive physical examination. The presence of a vascular tumor, such as a hemangioma, is a key clinical finding suggestive of KMS.
Laboratory tests: Blood tests are essential to confirm the diagnosis of KMS. The most important laboratory finding is a significantly low platelet count, often below 50,000 per microliter. Additionally, tests may reveal abnormal coagulation parameters, such as prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT). These abnormalities indicate consumptive coagulopathy, a hallmark of KMS.
Imaging studies: Various imaging techniques are employed to visualize the vascular tumor and assess its characteristics. Ultrasonography is commonly used as an initial imaging modality to evaluate the size, location, and blood flow within the tumor. Magnetic resonance imaging (MRI) or computed tomography (CT) scans may be performed for a more detailed assessment of the tumor's extent and involvement of adjacent structures.
Biopsy: In some cases, a biopsy of the vascular tumor may be necessary to confirm the diagnosis of KMS. A small sample of the tumor is obtained and examined under a microscope to identify its specific characteristics and rule out other potential causes of thrombocytopenia and coagulopathy.
It is important to note that the diagnosis of KMS requires the presence of all three components: a vascular tumor, thrombocytopenia, and consumptive coagulopathy. The healthcare team will carefully evaluate the clinical and laboratory findings, along with imaging results, to establish an accurate diagnosis and initiate appropriate management strategies.
