Kasabach–Merritt Syndrome

Abstract

The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.

Evans syndrome: clinical perspectives, biological insights and treatment modalities

“…There are some diseases with similar characteristics to ES. Main differential diagnoses are as follows: hemolytic uremic syndrome, characterized by microangiopathic hemolytic anemia, thrombocytopenia, leukocytosis, negative DAT test, clinically manifested as diarrhea, vomiting, fever, pallor, acute renal failure in children predominantly secondary to infections with Escherichia coli , Shigella dysenteriae or Streptococcus pneumoniae ; 13 thrombotic thrombocytopenic purpura, a disease associated with a functional deficit of ADAMTS 13 presenting as microangiopathic hemolytic anemia, thrombocytopenia, fever and neurological or kidney impairment; 14 Kasabach–Merritt syndrome, a rare disease predominant in early infancy with a high mortality rate of about 30% characterized by microangiopathic hemolytic anemia, thrombocytopenia, low fibrinogen levels, increment of D-dimer and giant hemangioma; 15 , 16 and paroxysmal nocturnal hemoglobinuria, a chronic life-threatening disease with hemolytic anemia, pancytopenia and thrombosis of vital organs, mainly in adults. These pathologies must be excluded because treatment is considerably different in each.…”

Features of the community–acquired pneumonia in a child with Kasabach-Merritt syndrome: case-report and mini-review

“…Every major hemangioma doesn't have to be accompanied by the development of KMS. The mortality rate is less than 10% in cases of skin lesions and reaches 60% in the presence of retroperitoneal tumors 8,10 . The exact etiology and pathogenesis of the disease are unknown, but in some cases, the hereditary pattern of this pathology has been discovered, autosomal dominant type 11 , although some papers deny this 12 .…”

Spontaneous Subdural Hematoma Associated with Kasabach-Merritt Syndrome: A Case Report

“…Kasabach-Merritt syndrome (KMS) is characterized by consumption coagulopathy (thrombocytopenia, anemia, hypofibrinogenemia, and high D-dimer level), angioma, and kaposiform hemangioendothelioma [1,2,3]. Herein we present the first reported case of spontaneous subdural hematoma associated with KMS.…”

“…Corticosteroid is considered the first-line therapy for KMS—either high-dose methylprednisolone (30 mg/kg for 3 d, followed by dose tapering) or oral corticosteroid (2–5 mg/kg per day), and intravenous corticosteroid may be more efficacious than oral corticosteroid [5]. Alfa-IFN-2a and chemotherapeutic agents are used to prevent tumor expansion [1,5]. Packed red cells, fresh frozen plasma, cryoprecipitate, and pentoxifyline may be considered for treatment [6].…”

“…Fresh frozen plasma (15 mL/kg) is recommended in patients with prolonged clotting time and generalized bleeding, and prior to invasive procedures [2]. Surgical excision, embolization, and radiotherapy can be used in appropriate patients [1,2]. The presented patient received medical therapy based on the clinical findings.…”