Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon

Shabtaie, Samuel A.; Wang, Bo; Owyong, Mark; Ruiz-Mesa, Catalina; Corrales-Medina, Fernando F.; Rojas, Claudia; Infante, Juan C.; Neville, Holly L.; Pérez, Eduardo A.; Sola, Juan E.; Hogan, Anthony R.

doi:10.1016/j.jpedsurg.2016.03.014

Cited by 9 publications

(9 citation statements)

References 11 publications

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“…Lesions involving the neck, viscera, retroperitoneum and mediastinum are associated with a particularly high mortality . The clinical management of these high‐risk KMP patients is highly challenging.…”

Section: Discussionmentioning

confidence: 99%

“…Lesions involving the neck, viscera, retroperitoneum and mediastinum are associated with a particularly high mortality. [26][27][28][29] The clinical management of these high-risk KMP patients is highly challenging. To date, no single treatment regimen has provided consistently reproducible results in terms of decreased tumor size and KMP symptom control.…”

Section: Discussionmentioning

confidence: 99%

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Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study

Chen

Xiang

et al. 2017

Intl Journal of Cancer

112

126

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Kaposiform hemangioendothelioma (KHE) is an aggressive disease with high morbidity and mortality. The aim of this study was to retrospectively evaluate the efficacy and safety of sirolimus for the treatment of progressive KHE. A multicenter, retrospective cohort study was conducted in patients with progressive KHE treated with sirolimus. A total of 52 patients were analyzed. Thirty-seven (71%) patients exhibited Kasabach-Merritt phenomenon (KMP) and were significantly younger than the patients without KMP [95% confidence interval (CI), 14.39-41.61; p < 0.001]. Patients without KMP were all treated with sirolimus alone, whereas 21 KMP patients with severe symptoms received short-term combination therapy with prednisolone. Overall, 96% and 98% of patients showed improved relief of notable symptoms and/or improved complications at 6 and 12 months after treatment, respectively. After sirolimus treatment, significant decreases in mean severity scores occurred at 6 months (95% CI, 2.23-2.54, p < 0.001) and 12 months (95% CI, 1.53-1.90, p < 0.001). Compared to KMP patients, patients without KMP showed a response that was similar to but less pronounced during the 12 months of treatment (95% CI, 40.87-53.80; p < 0.001). For subgroup analysis of KMP patients, there were no significant differences in tumor shrinkage between those treated with combination therapy and those receiving sirolimus alone (95% CI, 18.11-25.02; p > 0.05). No patients permanently discontinued treatment due to toxicity-related events, and no drug-related deaths occurred. Sirolimus was effective and safe for the treatment of progressive KHE. Sirolimus may be considered as a first-line therapy or as part of a multidisciplinary approach for the treatment of KHE.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study

Chen

Xiang

et al. 2017

Intl Journal of Cancer

112

126

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“…Deep tissue KHE are less common than cutaneous lesions, and reports of isolated splenic KHE are exceedingly rare, with only two pediatric cases and two adult cases reported. [11][12][13][14] Multifocal KHE has been reported as a simultaneously cutaneous and deep tissue structure, further illustrating the wide range of possible presentations. 6,15,16 Out of these four reports of isolated splenic KHE, only Shabtaie et al's description of a prenatally diagnosed splenic mass was associated with KMP.…”

Section: Discussionmentioning

confidence: 95%

“…6,15,16 Out of these four reports of isolated splenic KHE, only Shabtaie et al's description of a prenatally diagnosed splenic mass was associated with KMP. 13 In all but one of these reports, the splenic lesions were diagnosed on imaging. Mota et al reported on splenic KHE in an adult who underwent splenectomy and was found to have additional KHE lesions in the liver and vertebrae not identified on original imaging.…”

Section: Discussionmentioning

confidence: 99%

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

et al. 2021

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A healthy 2-year-old male with a history of mild persistent asthma visited the outpatient hematology clinic to evaluate intermittent epistaxis. There was no history of recent illness. His exam was notable for left upper extremity bruising secondary to recent venipuncture, but no lymphadenopathy or hepatosplenomegaly. Complete blood count (CBC) revealed hemoglobin 9.3 g/dL, leukocyte count 20.7 10 9 /L, and platelet count 33 k 10 9 /L. Peripheral blood smear exhibited polychromasia, numerous large platelets, and a few reactive lymphocytes.The differential diagnosis for both thrombocytopenia and anemia include processes leading to impaired hematopoiesis, increased cell destruction, or abnormal cell pooling. Impaired hematopoiesis could stem from infectious suppression, infiltrative processes (leukemia, metabolic disorders, etc.), or inherited and acquired bone marrow failure syndromes. Possible destructive or consumptive processes included microangiopathy, disseminated intravascular coagulation (DIC), or immune destruction. Sequestration within the liver or spleen was considered, but he was without hepatosplenomegaly to suggest a site of abnormal pooling. We approached his work-up with a stepwise approach. Screening tests were normal for common infectious diseases implicated in marrow suppression (Table 1). Since the patient appeared well and did not exhibit any findings that would prompt an immediate evaluation for malignancy or sepsis, we decided to repeat a short interval CBC to see if his cytopenias self-resolved. When they did not, further evaluation of the bone marrow was warranted. Bone marrow biopsy and aspirate did not demonstrate marrow infiltration or failure (Table 1). Lab work was sent to investigate consumptive processes. Lactate dehydrogenase levels and reticulocyte counts were elevated (Table 1), suggesting a hemolytic process. A hemoglobinopathy screen and glucose-6-phosphate dehydrogenase testing were normal, though active hemolysis can skew results. In an ill-appearing child, the presence of hemolysis and thrombocytopenia should prompt an investigation for DIC or microangiopathy, but in this well appearing-child, an immune process was thought likelier. We, therefore, pursued an immunologic work-up, including screening for autoimmune lymphoproliferative syndrome (ALPS) (Table 1). The results were unrevealing except for a low IgG level at 228 (505-1280 mg/dL) with normal lymphocyte subsets.Low IgG is often associated with systemic autoimmune disorders, vasculitides, atopy, and asthma. We attributed the low IgG and eosinophilia to the patient's known history of asthma. A consumptive autoimmune process was high on the differential, and the involvement of two cell lines would be classified as Evans syndrome. Evans syndrome, though never a primary diagnosis, is treated upfront with

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“…Approximately 70% of KHE cases are associated with thrombocytopenia, coagulation factor depletion and severe anemia, known as the Kasabach-Merritt phenomenon (KMP) (3). In recent years, multiple lesions and complex cases of KHE have been increasingly reported (4)(5)(6)(7)(8). However, there is currently no appropriate assessment of quality of life (QOL) in patients with KHE.…”

Section: Introductionmentioning

confidence: 99%

Health-Related Quality of Life in Children With Kaposiform Hemangioendothelioma

et al. 2022

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Background and ObjectiveKaposiform hemangioendothelioma (KHE) is a rare, aggressive and borderline vascular tumor mainly occurring in infants and children. The aim of this study was to determine the health-related quality of life (HRQOL) in children with KHE.MeasuresA total of 91 children with KHE participated in this cross-sectional study. The HRQOL was assessed by the age-specific Pediatric Quality of Life Inventory Version 4.0 (PedsQL™ 4.0) Infant Scales, Family Information Form (FIF), Family Impact Module (FIM) and Generic Core Scales (GCS). For comparison, demographically matched healthy children were recruited as a control group. The main outcome measure of HRQOL was analyzed in the two groups. We determined related factors that influenced the HRQOL in children with KHE and their parents by using a stepwise multiple regression analysis.ResultsThe study found that the scores of each item in the family impact module (FIM) were lower than 75, which suggesting that KHE can make the parents of patients in a state of poor quality of life. The scores of physiological and psychosocial domains in all age groups of patients with KHE were lower than those of normal children (P < 0.01). Activity dysfunction is the factor influencing the physiological function score of all-age patients. KMP is the factor influencing the psychosocial function score of all-age patients.ConclusionsThe findings presented here suggest that patients with KHE have a poor HRQOL. KMP and activity dysfunction are risk factors for poor HRQOL in patients with KHE. However, lesion size, lesion location and education level of the mother and father were not related to the HRQOL.

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Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon

Cited by 9 publications

References 11 publications

Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study

Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

Health-Related Quality of Life in Children With Kaposiform Hemangioendothelioma

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