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New Case Report Documents Successful Treatment Approach to a Very Rare Form of Splenic Kaposiform Hemangioendothelioma

January 4, 2022

In December 2021, a multidisciplinary team of clinicians from UPMC Children’s Hospital of Pittsburgh Divisions of Pediatric Hematology/Oncology and Pathology published a new case report on a rare type of splenic kaposiform hemangioendothelioma in the American Journal of Hematology.

Deirdre Nolfi-Donegan, MD, from the Division of Pediatric Hematology/Oncology, was the paper's senior author documenting the team’s approach to identifying and treating this rare form of hemangioendothelioma in a 2-year-old male. Former Division fellow Matthew Shiel, MD, and Marti Goldenberg, MD, a current pediatric resident in the Division, were the co-first authors of the case report. Ramasubramanian Kalpatthi, MD, director of Pediatric Sickle Cell Research, was the patient's primary hematologist. Subramanian Subramanian, MD, from the Department of Radiology, and Miguel Reyes-Múgica, MD, Chief of the Division of Pediatric Pathology and the Marjory K. Harmer Endowed Chair in Pediatric Pathology were instrumental collaborators on the case with tissue analysis and image acquisition and interpretation, respectively, and are co-authors of the report.

Kaposiform hemangioendotheliomas (KHE) are very rare vascular types of tumors that, in most cases, arise in cutaneous or deep tissue structures. These types of tumors were only very recently first described in the literature – in 1989. Because of the exceptional rarity of KHE in all of its forms so far identified, there are no standard treatment guidelines available for clinicians to follow, and long-term outcomes of the disease are unknown or unpredictable due to the small number of documented presentations.

In this instance, the patient first presented with a type of consumptive coagulopathy for which the team was unable to initially find a cause. Using a broader differential diagnosis, the team explored the possibilities of an autoimmune disorder or a vascular malformation, but the only real abnormalities noticed were with the patient's spleen, which exhibited massive splenomegaly and cystic lesions. However, imaging studies were inconclusive.

“Our case was unique in a couple of ways," says Dr. Nolfi-Donegan. "The isolated existence of a kaposiform hemangioendothelioma in the spleen is virtually unheard of. We could only find a handful of cases documented in the literature, and only two of which were in pediatric-age patients. Furthermore, to have a patient present with bleeding and coagulopathy symptoms also is a rare presentation. Typically in this type of lesion, there is some type of skin or cutaneous clue that is indicative of something lurking beneath the surface or in deeper organ tissue. However, in this case, our patient had no skin lesion. We only had abnormal findings related to the spleen, but imaging studies were inconclusive.”

The patient’s condition deteriorated over several weeks; his coagulopathy and bleeding continued to be unresponsive to treatment, and his spleen continued to show gross abnormalities. For the team, it was clear that his spleen would need to be removed and tissue analysis conducted to try and identify an underlying cause.

However, the team was extremely concerned about the surgical procedure because of the patients uncontrolled coagulopathy and bleeding. Surgery on this patient would be far from routine. In order to mitigate the surgical risks, the patient was moved to the intensive care unit for a 24-hour period prior to the surgery, where he was aggressively treated with continuous infusions of fresh-frozen plasma, as well as infusions of CPP and pooled platelets. Fortunately, the patient experienced no excess bleeding during the procedure and recovered rapidly.

It was not until the patient's spleen was removed during surgery that the team was able to achieve confirmation, through tissue analysis, that there existed a diffuse KHE within the spleen and the cause of the patient’s symptomatic coagulopathy.

“Once the patient’s spleen was removed, his condition almost immediately improved. His coagulopathy and bleeding resolved, while laboratory values returned to within normal reference ranges,” says Dr. Nolfi-Donegan. “This case is an exemplification of how multidisciplinary care can benefit patient care and solve a complex clinic presentation. It took a concerted effort by our team of hematologists, surgeons, and radiology, pathology clinicians to resolve this case with a good outcome.”

Learn More

To learn more about the details of Dr. Nolfi-Donegan's teams' unique case and the diagnostic and treatment approaches they applied to manage the patient successfully, pediatric hematology/oncology physicians can read the entire case report and discussion at the following link.


Goldenberg M, Shiel M, Subramanian S, Kalpatthi R, Reyes-Múgica M, Nolfi-Donegan D. Splenic Kaposiform Hemangioendothelioma Presenting as Insidious Consumptive Coagulopathy. Am
J Hematol
. 2021; 1-7. doi:10.1002/ajh.26370.