Diffuse Infiltrative Hemangioma of Pancreas Accompanied by Kasabach-Merritt Syndrome: A Case Report
Kasabach-Merritt syndrome (KMS) is a rare and life-threatening condition associated with aggressive vascular tumors such as Kaposiform hemangioendothelioma and tufted angioma. It is characterized by thrombocytopenia and intravascular coagulation, which can occasionally progress to disseminated intravascular coagulation. The syndrome is particularly challenging to diagnose and manage, especially when it involves visceral organs like the pancreas. This case report presents a rare instance of diffuse infiltrative hemangioma (DIH) of the pancreas accompanied by KMS, highlighting the diagnostic and therapeutic approaches that led to a successful outcome.
The patient in this case was a 1.5-month-old male infant who was admitted to the hospital due to scleral icterus and jaundice across his entire body. Initial laboratory tests revealed anemia, thrombocytopenia, coagulopathy, and abnormal liver function. Clinical examination showed that the infant’s skin was dark yellow with visible petechiae. An abdominal ultrasound scan was performed, which revealed three key findings: the liver was enlarged by 3.3 cm, the common bile duct was dilated, and the pancreas showed diffuse swelling with inhomogeneous rough echogenicity, heterogeneous intra-parenchymal texture, and abundant vessels. These findings were further confirmed by enhanced computed tomography (CT), which also showed diffuse swelling of the entire pancreas with abundant vessels. Based on these findings, a diagnosis of DIH of the pancreas accompanied by KMS was made.
The infant was initially treated empirically with prednisone at a dose of 1 mg/kg intravenously. However, after one week of treatment, there was no significant improvement in the size of the pancreas or the coagulation status. Additionally, the platelet count dropped to 3 × 10^9/L, although there were no signs of bleeding. Given the lack of response to prednisone, the therapeutic regimen was changed to sirolimus. After one month of sirolimus treatment, the infant’s jaundice and petechiae showed significant improvement. Both the coagulation indices and liver function returned to normal, although the platelet count remained low. After four months of sirolimus treatment, the platelet count normalized, and an abdominal ultrasound scan showed no significant changes in the size or morphology of the pancreas, but the internal echo became slightly finer and more homogeneous. After one year of treatment, all laboratory tests returned to normal, and there was no recurrence of thrombocytopenia. A repeat abdominal ultrasound scan showed that the affected pancreas had essentially returned to its normal size, and the blood flow within the pancreas was significantly reduced. These results were further confirmed by enhanced CT scans. Throughout the two-year treatment period with sirolimus, no side effects were observed, and the infant continues to receive maintenance treatment with sirolimus.
KMS lesions are typically cutaneous and solitary hemangiomas, and they rarely occur in visceral organs or bones. The syndrome is most commonly diagnosed within the first year of life, and the onset age in this case was 1.5 months. Hemangiomas in the pancreas are extremely rare, and due to their internal location and lack of specific clinical manifestations, they are difficult to diagnose. Pathologic biopsy is often not feasible due to the high risk of bleeding, especially in critically ill infants or when the tumor is located in a sensitive area like the pancreas. While CT and magnetic resonance imaging (MRI) are well-established methods for evaluating pancreatic lesions, they have some limitations. Ultrasound, on the other hand, offers several advantages, including portability, low levels of radiation, and applicability to critically ill patients who cannot undergo other examinations. Importantly, ultrasound can clearly show the size, shape, and internal echo of the pancreas, as well as changes in blood flow signals within the organ. Therefore, ultrasound is considered a preferential tool for the preliminary screening of pancreatic hemangioma accompanied by KMS.
There is no standard guideline for treating large hemangiomas accompanied by KMS. However, sirolimus has shown promising results in the treatment of such cases. In this case, after one year of sirolimus treatment, the infant showed stable vital signs, and all laboratory indicators, as well as the size of the pancreas, returned to normal. This suggests that sirolimus is effective in the treatment of hemangioma with KMS, although further prospective trials are needed to assess the efficacy of this drug and to determine the optimal therapeutic dose and duration of treatment.
In conclusion, this case report describes a very rare instance of DIH of the pancreas accompanied by KMS. Ultrasound was used for early diagnosis, and sirolimus was employed for early treatment, resulting in a good therapeutic outcome. When an ultrasound scan reveals diffuse swelling of the pancreas and abundant blood flow signals, along with abnormal hematologic parameters, pancreatic diffuse hemangioma accompanied by KMS should be considered. Ultrasound is a preferential tool for the definite diagnosis of patients with DIH of the pancreas accompanied by KMS, especially in infants, and it is also a simple tool for prolonged follow-up.
doi.org/10.1097/CM9.0000000000001037
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