Kasabach-Merritt syndrome

Kasabach-Merritt syndrome (KMS), also known as hemangioma-thrombocytopenia syndrome, is a rare disorder characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, and subsequent consumptive coagulopathy in association with vascular tumors, particularly kaposiform hemangioendothelioma or tufted angioma.



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Narrow down the case reports



Total: 198 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(53.8%)
8002725
[Kasabach-Merritt syndrome with pancreatic hemangioma in an infant].
Goldszmidt D, Pariente D, Yandza T, Dubousset AM, Valayer J.
Arch Fr Pediatr. 1993;50(7):593-7.
Jaundice Hepatosplenomegaly Reticulocytosis
IFNA2
Hemangioendothelioma Homo sapiens Infant, Newborn Male Pancreatic Neoplasm Syndrome Thrombocytopenia Time Factors
2
(52.7%)
18822934
Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child.
Tang JY, Chen J, Pan C, Yin MZ, Zhu M.
World J Pediatr. 2008;4(3):227-30.
Petechiae Splenomegaly Anemia
FGB
Blood Coagulation Disorders Child, Preschool Disseminated Intravascular Coagulation Hemangioma, Cavernous Homo sapiens Magnetic Resonance Imaging Male Splenectomy Splenic Neoplasms Syndrome Thrombocytopenia X-Ray Computed Tomography
3
(52.0%)
24708716
(4016776)
Case report: living donor liver transplantation for giant hepatic hemangioma using a right lobe graft without the middle hepatic vein.
Zhong L, Men TY, Yang GD, Gu Y, Chen G, Xing TH, Fan JW, Peng ZH.
World J Surg Oncol. 2014;12:83.
Jaundice Ascites Anemia
Adult Females Graft Rejection Hemangioma Hepatectomy Homo sapiens Living Donors Male Middle Aged Postoperative Complications X-Ray Computed Tomography
4
(46.4%)
1561152
Consumptive coagulopathy in utero associated with multiple vascular malformations.
Pierce RN, Dunn L, Knisely AS.
Pediatr Pathol. 1992;12(1):67-71.
Edema Cholestasis Ascites Pulmonary hemorrhage
Blood Vessel Cardiovascular Diseases Disseminated Intravascular Coagulation Females Hemangioma Homo sapiens Infant, Newborn Male Pregnancy Pregnancy Complications, Cardiovascular Thrombosis Vitamin K Deficiency Bleeding
5
(43.5%)
24575011
(3934804)
Chemotherapy and surgical approach with repeated endovascular embolizations: safe interdisciplinary treatment for kasabach-merritt syndrome in a small baby.
Nakib G, Calcaterra V, Quaretti P, Moramarco LP, Bonalumi G, Brunero M, Pelizzo G.
Case Rep Oncol. 2014;7(1):23-8.
Thrombocytopenia Anemia Pelvic mass
5
(43.5%)
10864058
Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia.
Pampin C, Devillers A, Treguier C, Fremond B, Moisan A, Goasguen J, Le Gall E.
J Pediatr Hematol Oncol. 2000;22(3):256-8.
Hemangioma Anemia Abdominal mass
Anemia Blood Platelets Hemangioma, Cavernous Homo sapiens Indium Radioisotopes Infant Male Radionuclide Imaging Splenectomy Splenic Neoplasms Syndrome Thrombocytopenia
7
(43.3%)
26943417
(4595412)
Obstructive jaundice caused by a giant liver hemangioma with Kasabach-Merritt syndrome: a case report.
Yano T, Kobayashi T, Kuroda S, Amano H, Tashiro H, Ohdan H.
Surg Case Rep. 2015;1(1):93.
Jaundice Thrombocytopenia Hyperbilirubinemia
FGB
8
(42.9%)
1237805
[Giant occipital hemangioendothelioma with thrombocytopenia, anemia and hypofibrinogenemia treated by total excision (author's transl)].
Nagashima C, Takahama M, Miyaji H, Maeda W, Matsuura R.
No Shinkei Geka. 1975;3(7):547-56.
Purpura Anemia
Afibrinogenemia Anemia Head and Neck Neoplasms Hemangioendothelioma Hemangioma Homo sapiens Infant Infant, Newborn Male Preoperative Care Syndrome
9
(42.5%)
25471004
Unusual treatment of Kasabach-Merritt syndrome secondary to hepatic hemangioma: embolization with bleomycin.
Bozkaya H, Cinar C, Unalp OV, Parildar M, Oran I.
Wien Klin Wochenschr. 2015;127(11-12):488-90.
Hemangioma Anemia Hepatic hemangioma
Adult Antibiotics, Antineoplastic Chemoembolization, Therapeutic Females Hemangioma Homo sapiens
9
(42.5%)
22175175
[Successful anesthetic management for resection of a giant hepatic hemangioma with Kasabach-Merritt syndrome using FloTrac system].
Wakabayashi S, Yamaguchi K, Kugimiya T, Inada E.
Masui. 2011;60(11):1326-30.
Hemangioma Microangiopathic hemolytic anemia Hepatic hemangioma
Blood Pressure Cardiac Output Disseminated Intravascular Coagulation Females General Anesthesia Hemangioma Homo sapiens Hydroxyethyl Starch Derivatives Intraoperative Care Monitoring, Intraoperative Stroke Volume
        

Phenotype(s) retrieved from Orphanet

    Total: 23

HPO ID Term Frequency
HP:0001028 Hemangioma Very frequent (99-80%)
HP:0001873 Thrombocytopenia Very frequent (99-80%)
HP:0011900 Hypofibrinogenemia Very frequent (99-80%)
HP:0000967 Petechiae Frequent (79-30%)
HP:0000979 Purpura Frequent (79-30%)
HP:0005306 Capillary hemangioma Frequent (79-30%)
HP:0012329 Tufted angioma Frequent (79-30%)
HP:0000975 Hyperhidrosis Occasional (29-5%)
HP:0001875 Neutropenia Occasional (29-5%)
HP:0001882 Leukopenia Occasional (29-5%)
HP:0001903 Anemia Occasional (29-5%)
HP:0001937 Microangiopathic hemolytic anemia Occasional (29-5%)
HP:0005520 Chronic disseminated intravascular coagulation Occasional (29-5%)
HP:0008151 Prolonged prothrombin time Occasional (29-5%)
HP:0031207 Hepatic hemangioma Occasional (29-5%)
HP:0100766 Abnormal lymphatic vessel morphology Occasional (29-5%)
HP:0000998 Hypertrichosis Very rare (4-1%)
HP:0001923 Reticulocytosis Very rare (4-1%)
HP:0002027 Abdominal pain Very rare (4-1%)
HP:0002098 Respiratory distress Very rare (4-1%)
HP:0003270 Abdominal distention Very rare (4-1%)
HP:0008069 Neoplasm of the skin Very rare (4-1%)
HP:0040213 Hypopnea Very rare (4-1%)


Phenotype(s) retrieved from case reports

    Total: 42

HPO ID Term # of case reports
HP:0001028 Hemangioma 64
HP:0001873 Thrombocytopenia 57
HP:0005521 Disseminated intravascular coagulation 11
HP:0031207 Hepatic hemangioma 11
HP:0001048 Cavernous hemangioma 9
HP:0001903 Anemia 6
HP:0001635 Congestive heart failure 4
HP:0002664 Neoplasm 4
HP:0005306 Capillary hemangioma 4
HP:0000952 Jaundice 3
HP:0001937 Microangiopathic hemolytic anemia 3
HP:0002098 Respiratory distress 3
HP:0007461 Hemangiomatosis 3
HP:0002797 Osteolysis 2
HP:0008151 Prolonged prothrombin time 2
HP:0100742 Vascular neoplasm 2
HP:0000126 Hydronephrosis 1
HP:0000790 Hematuria 1
HP:0001396 Cholestasis 1
HP:0001510 Growth delay 1
HP:0001722 High-output congestive heart failure 1
HP:0001876 Pancytopenia 1
HP:0001878 Hemolytic anemia 1
HP:0001882 Leukopenia 1
HP:0001945 Fever 1
HP:0001971 Hypersplenism 1
HP:0002045 Hypothermia 1
HP:0002170 Intracranial hemorrhage 1
HP:0002176 Spinal cord compression 1
HP:0002576 Intussusception 1
HP:0002721 Immunodeficiency 1
HP:0002756 Pathologic fracture 1
HP:0002835 Aspiration 1
HP:0002904 Hyperbilirubinemia 1
HP:0003072 Hypercalcemia 1
HP:0003470 Paralysis 1
HP:0005214 Intestinal obstruction 1
HP:0011900 Hypofibrinogenemia 1
HP:0020110 Bone fracture 1
HP:0031500 Abdominal mass 1
HP:0100806 Sepsis 1
HP:0410266 Visceral hemangioma 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID