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Kaposiform Hemangioendothelioma with Kasabach-Merritt Syndrome-right arm treated with steroids and propranolol
Source publication
A case series of four patients who presented with large surface vascular tumors and low platelet count and their management is reported. Medical management was done with steroids, propranolol and vincristine in different combinations. The final response was excellent without surgery.
Contexts in source publication
Context 1
... was thrombocytopenia (60,000) and the coagulation profile was deranged [ The baby was initially treated with steroids but the platelets dropped to 13,000 cells/mm, and hence was started on platelet transfusions. After transfusion of two units of platelet concentrates, the swelling appeared to shrink, but the platelet count dropped to 10,000 and the baby developed small purpuric spots over trunk and a subcutaneous hematoma (3 × 3 cm) over the left calf. A possibility of coagulopathy aggravated by repeated platelet transfusion was considered. Hence, platelet transfusions were discontinued and propranolol was started. From the 10 th day, the swelling started regressing and the platelet count started to rise. The baby was subsequently discharged on oral steroids and propranolol. After a week, there was a steady rise in count (platelets 70,000 cells/mm). Propranolol was tapered after a month and steroids were continued and then tapered. The hemangioma slowly diminished in size. When last seen at 1 year of age, there was faint skin staining at the site of the original hemangioma [ Figure ...
Context 2
... full-term male baby was referred at 7 hours of life with a large swelling involving the right arm. The antenatal scans were normal. On examination, there was an 8 × 10 cm, red, tense, shiny, warm swelling over the right shoulder, extending to the upper two-thirds of the right arm [ Figure 3]. There was no distal neurovascular deficit. Systemic examination was normal. A diagnosis of KHE/fibrosarcoma was ...
Similar publications
Kasabach-Merritt syndrome (KMS) is characterized by a rapidly enlarginghemangioma, thrombocytopenia, microangiopathic hemolytic anemia and consumption coagulopathy as a result of platelet and red blood cell trapping and activation of clotting system within the vasculature of hemangioma. This syndrome is shown to be associated with kaposiform hemang...
Rationale
Kaposiform hemangioendothelioma (KHE) is an aggressive vascular tumor, mainly occurring in infants and young children and previously reported cases were mainly cutaneous or visceral form. Intestinal kaposiform hemangioma was first reported in 2012. Intestinal type KHE showed better prognosis if the lesion was limited in the gastrointestin...
Kasabach-Merritt Phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy in the presence of an enlarging vascular lesion. The syndrome usually develops in infancy and is associated with a high morbidity and mortality rate. We report a case of successful management of refractor...
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that...
Background: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and of the first decade of life. It is locally aggressive and potentially life threatening when associated to consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of...
Citations
... At the same time, the turbulence of the blood flow, determined by the abnormal vascularization of the mass, causes the formation of micro-clots with secondary consumption of coagulation factors and mechanical destruction of red blood cells. KSP is a lifethreatening condition with a reported mortality rate of 10-40% [19]. ...
Objective. To describe the case of a large cervical mass diagnosed in the late third trimester with development of Kasabach-Merritt phenomenon (KMP) in the immediate postnatal period, along with a literature review.
Methods. Description of case-report and literature search through Medline/Pubmed, performed from inception to December 2022 for articles relating to the pre and postnatal diagnosis of KMP.
Results. A 36-year-old multiparous woman was admitted to hospital for contractions at 40 weeks of gestation, in an otherwise uneventful pregnancy. Admission’s ultrasound showed the presence of a voluminous mass of 14x15 cm of the posterior side of the neck, highly vascularized, and no signs of hemodynamic imbalance. Postnatally, blood tests showed the presence of severe anemia and thrombocytopenia requiring several transfusions of blood, plasma, platelets and clotting factors. Due to the association of congenital hemangioma and thrombocytopenia a diagnosis of KMP was made. After attempts of conservative treatment, surgical removal was needed to stop the hematological cascade with regression of symptoms. The review of the literature identified 14 articles including 9 cases of prenatally suspected KMP and 6 diagnosed in the immediate postnatal period and without signs of fetal hydrops. Adverse perinatal outcome, in terms of postnatal death/termination of pregnancy, was observed in 67% of cases (6/9) in the prenatally suspected group and 33% of cases in those with a postnatal diagnosis of KMP. Fetal hydrops was present in 83% of cases with adverse perinatal outcome.
Conclusions. The Kasabach-Merrit syndrome is a rare condition, which can have a dangerous evolution when it develops in utero or in the immediate postnatal period carrying a risk of perinatal mortality of approximately 50%. Even if the fetus shows no signs of anemia or heart failure, the risk of developing it in the immediate postnatal period is high and should be mentioned to the couple.
... [1][2][3][4] It was first described in 1940 in a male infant; only approximately 200 cases have been reported in the literature, most of which (80%) are diagnosed within the first year of life. 5,6 The mortality rate of KMS is 20%-40%, mainly because of intractable bleeding caused by the consumption coagulopathy. 4,7 The lack of consensus to guide therapy makes the management much more challenging. ...
Kasabach-Merritt syndrome is a rare but life-threatening disease in which a rapidly growing vascular tumor induces localized intravascular coagulation, causing thrombocytopenia, microangiopathic hemolytic anemia, and consumption coagulopathy. It presents mainly in infants and young children. We present an adult with recurrent and severe lower gastrointestinal bleeding due to Kasabach-Merritt syndrome, treated successfully with sirolimus after multiple other failed interventions.
... Epidemiological surveys have shown that the incidence of KHE is approximately 0.71/100,000 in children (1). KHE can be accompanied by a severe complication known as the Kasabach-Merritt phenomenon (KMP), which is characterized by thrombocytopenia, hypofibrinogenemia, and clotting factor consumption (2)(3)(4)(5). According to the morphology and the clinical manifestations, Ji (6) divided KHE into three types, namely, superficial (which is limited to the skin and subcutaneous soft tissue without the invasion of muscles and bones, chest and abdominal cavity), mixed (which involves both the skin and subcutaneous deep muscles, bones and/or joints) and deep (which has no skin manifestations). ...
Introduction: Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumor that mostly appears in infants and adolescents. KHE with spinal involvement is extremely rare. The aim of this study was to review the imaging features, clinical manifestations and treatment of KHE patients with spinal involvement.
Materials and Methods: We reviewed patients with KHE who were admitted to Pediatric Surgery of West China Hospital of Sichuan University from April 2014 to August 2020, and the cases were evaluated.
Results: Seven patients with spinal involvement were enrolled in the study, including four (57.1%) males and three (42.9%) females. The age at onset ranged from 1.0 day to 4.0 years, with an average of 1.6 years. Five (71.4%) had pain due to bone destruction, three patients (42.9%) had decreased range of motion (ROM), four (57.1%) patients had scoliosis, two (28.6%) patients developed claudication, and three patients (42.9%) presented with a soft tissue mass in the neck of the back. Five patients (71.4%) had the Kasabach-Merritt phenomenon (KMP), with a minimum platelet value of 8 × 10 ⁹ /L. All patients were treated with sirolimus, and showed regression of the lesion and/or normalization of the hematologic parameters.
Conclusion: KHE with spinal involvement is difficult to diagnose due to its rarity and variable symptoms, which need to be recognized to start early treatment. The management of KHE with spinal involvement should be performed by a multidisciplinary team. Sirolimus can improve outcomes in patients with KHE with spinal involvement.
... The vascular tumors are one of the most common neoplasms in children 1,2 . Despite the skin hemangiomas' multiplicity and size, their diagnosis does not cause significant difficulties, and the benign course leads to a favorable prognosis 1,3,4 . ...
... Involution of the tumor and correction of life-threatening coagulopathy are the main objectives of the treatment of Kasabach-Merritt syndrome 3 . Currently, different options of treatment are suggested, which include pharmacological therapy, vascular embolisation, radiation therapy and surgical removal of the tumor 3,6,7,12,18,19 . ...
... Despite the radicality of the previously described treatment modalities, the most commonly used are drugs which facilitate the hemangioma involution (Table 1) 20 . Hormonotherapy with corticosteroids for the Kasabach-Merritt syndrome in children should be considered as a primary treatment method, if the surgery cannot be performed 3,17,20,21,23 . However, these drugs effectiveness, as indicated by most researchers, is ambiguous 6,19 . ...
Introduction. The Kasabach-Merritt syndrome is a rare and severe disease that occurs in children in the presence of a large-sized hemangioma, accompanied by coagulation disorders. This disease complicates the course of many somatic diseases in children, including community-acquired pneumonia, due to the clotting disorder.Case presentation. A case of a 4.5-month-old child with a combination of capillary hemangioma of the dextral chest surface, complicated by Kasabach-Merritt syndrome, and community-acquired right-sided focal pneumonia is presented. The possible effect of the Kasabach-Merritt syndrome on the course of com-munity-acquired pneumonia has been analyzed. Additionally, the drugs’ effect on the coagulation disorder, the state of hemangioma, and the course of community-acquired pneumonia in children have been investigated.
... When examining the right breast, it propranolol. An unrecognized syndrome, without adequate therapy, almost certainly leads to a very fast lethal outcome 9,10 . ...
Introduction. Primary breast angiosarcoma is a very rare tumor and accounts
for 0.04% of all breast malignant tumors and most commonly occur in young
women. Kasabach-Merritt syndrome (KMS) is described as consumption
coagulopathy with thrombocytopenia, and without adequate therapy almost
certainly leads to a very fast lethal outcome. Case report. We present a
rare case of 60-year-old postmenopausal woman with metastatic primary
angiosarcoma of the breast associated with a picture like Kasabach-Merritt's
syndrome (thrombocytopenia and anemia without the coagulation factor
disorder with massive bleeding in the tumor). Conclusion. Primary breast
angiosarcoma in postmenopausal women is a very rare tumor, and may be
associated with anemia and thrombocytopenia without other laboratory
parameters for Kasabach-Merritt's syndrome. Anemia and thrombocytopenia are
refractory to standard treatment protocols, and also significantly reduces
the quality of life of these patients. The literature contains only a few
cases of Passociated with thrombocytopenia or with KMS and there are no
clear defined protocols for the treatment of these patients, which requires
the presentation of as many cases as possible.
... About 80% of the cases develop this phenomenon within the 1 st year of life with a reported mortality of 10%-37% even after treatment. [6,7] It shows an unpredictable and variable response to conventional pharmacological drugs, such as vincristine, propranolol, steroids, or interferons. Surgical excision is not feasible in every case. ...
Kasabach-Merritt Phenomenon (KMP) is a potentially life-threatening condition characterised by thrombocytopenic consumptive coagulopathy. KMP is almost exclusively associated with two uncommon vascular tumours, Kaposiform haemangioendotheliomas (KHE) and tufted angiomas (TA). It is clinically characterized by a rapid increase in the size of the pre-existing vascular plaque and deranged blood coagulation profiles. Early recognition and treatment is of crucial importance. Hemodynamic stability should be achieved as soon as possible with the use of fresh frozen plasma infusions. Surgical excision and embolization, when feasible, are the line of treatment. Oral prednisolone with or without vincristine are most commonly used and effective treatment. Oral sirolimus has shown its efficacy and safety in recent reports and-series. Propranolol and anti-platelet drugs are commonly used as a second-line therapy. Other drugs and combination therapies are used in non-responsive recalcitrant cases.
... De Pree (1963) reviewed 49 cases of giant hemangiomas with thrombocytopenia and found great variation in the localization and histological, clinical course, and type of treatment in them (27). Arunachalam et al., (2012) reported a case series of four patients who presented with large surface vascular tumors and low platelet count and their management. They did medical management with steroids, propranolol and vincristine in different combinations. ...
... They did medical management with steroids, propranolol and vincristine in different combinations. Their results showed excellent final response without surgery (37). It is concluded that KMS is a complicated disease with high mortality and difficult treatment modalities. ...
Cavernous hemangioma of the colon is a rare vascular malformation but is clinically important because it can sometimes cause massive bleeding. We report a case of thrombocytopenia in a post-partum young woman with large cavernous hemangioma of the ileum and colon, culminating in Kasabach Merritt syndrome (KMS), massive uncontrollable hemorrhage, and death. CT scan shows multiple submucosal bulgings in all segments of the colon. Macroscopic examination showed multiple well-defined masses, with gray-brown color in ileum, cecum and ascending colon. The masses were found to be a cavernous hemangioma with a thick fibrotic wall and extensive intra-cystic hemorrhage. KMS is an uncommon complication of colonic large hemangiomas that, as in our patient, can lead to uncontrollable bleeding and death and should be kept in mind by visceral surgeons as one differential diagnosis of large intra-abdominal tumorous masses, especially in young adults. © 2018 Tehran University of Medical Sciences. All rights reserved.
... The major indications for propranolol treatment in infantile hemangiomas are described in Table 3. [33][34][35][36][37] Mechanism of action Different mechanisms of action have been proposed for the efficacy of propranolol in infantile hemangiomas viz 1. ...
... Antihypertensives which act as direct vasodilators (nitrates, calcium channel blockers) and those which inhibit vasoconstriction (angiotensin receptor blockers, alpha adrenergic Kassabach-Merritt phenomenon associated with kaposiform hemangioendothelioma, tufted angioma -propranolol in combination with vincristine or steroids 36,37 PHACES: Posterior fossa defects, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal clefting or supraumbilical raphe, IH: Infantile hemangiomas receptor blockers) have been tried in the medical management of Raynaud's phenomenon. Dihydropyridine calcium channel blockers are by far the most commonly studied and prescribed class of agents for Raynaud's phenomenon. ...
Hypertension is a global health problem. Antihypertensives are the mainstay of treatment for hypertension. Some of them were accidentally found to be useful in alopecias and infantile hemangiomas and have now become standard treatment for these conditions as well. Antihypertensives are also being studied for other dermatological indications, where they have shown promising efficacy. This review focuses on the dermatological indications for antihypertensives, discussing the drugs that have been tried, as well as their efficacy, dosage, duration of therapy, and adverse effects.
... Biopsy of the lesion was deferred as trauma has been implicated as a perpetuating factor. 1 Kasabach-Meritt phenomenon is a potentially life-threatening condition characterized by thrombocytopenic consumptive coagulopathy with a reported mortality of 20-30%. 2,3 This phenomenon is almost exclusively associated with Kaposiform hemangioendotheliomas and tufted angiomas. 4 Abnormal platelet activation and aggregation, secondary to interaction with abnormal tumor endothelium, results in localized trapping of the platelets. ...
... KMS is a rare complication associated with hemangiomas, and has a mortality rate of 20%-30% during the first few weeks of life [11]. The phenomenon also arises from kaposiform hemangioendothelioma (KH), kaposiform lymphatic anomaly, and tufted angioma (TA), and some malignant neoplasms such as angiosarcoma [12]. ...
Patient: Male, 1
Final Diagnosis: Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh
Symptoms: Gross hematuria
Medication: —
Clinical Procedure: CT-guided drainage • blood transfusion
Specialty: Hematology
Objective
Rare disease
Background
Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh.
Case Report
A 33-year-old Taiwanese male presented with a painful 20-cm mass over his right thigh and gross hematuria for 2 days. Hemangiomatosis was bioptically proven in infancy and the patient was under regular follow-up. Physical examination revealed normal heart rate, respiratory rate, and body temperature. Multiple palpable lumps with brown and purple areas of skin over the neck, trunk, and right thigh were noted. Laboratory examinations revealed thrombocytopenia anemia and elevated fibrin degradation products. There were no signs of sepsis. Blood transfusion and steroid therapy were executed. Computed tomography showed a huge complicated subcutaneous hematoma in the right thigh. Drainage of the huge hematoma was performed and antibiotics were prescribed. After the local infection in the right thigh and the bleeding tendency were controlled, the patient was discharged in a stable condition two weeks later.
Conclusions
A huge infected hematoma and widespread hemangiomas are extremely rare complications of Kasabach-Merritt syndrome. There are no known treatment guidelines currently available. Our patient was successfully treated with steroids, drainage, and antibiotics.
