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Kasabach‑Merritt Syndrome Combined with Hypercalcemia: a Case Report

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Kasabach‑Merritt Syndrome Combined with Hypercalcemia: a Case Report 6164 EXPERIMENTAL AND THERAPEUTIC MEDICINE 14: 6164-6168, 2017 Kasabach‑Merritt syndrome combined with hypercalcemia: A case report RUNYING ZOU, FANG PENG, TIAN YU, SAIZHEN ZENG, YALAN YOU, KEKE CHEN, HUI ZOU, XIN TIAN, CHENGGUANG ZHU and XIANGLING HE Department of Hematology and Oncology of Children's Medical Center, Hunan Provincial People's Hospital, Changsha, Hunan 410005, P.R. China Received November 4, 2016; Accepted August 4, 2017 DOI: 10.3892/etm.2017.5332 Abstract. The present case report presented the diag- malformations, and both may cause Kasabach-Merritt nosis and treatment course of an infant diagnosed with syndrome (KMS) (2‑4). KMS was firstly reported in 1940 Kasabach-Merritt syndrome (KMS) combined with hyper- by Kasabach and Merritt (5), and is a relatively rare disease calcemia (HC). A 35-day-old infant with swelling on the accounting for only 1% of all hemangiomas (6). Hemangiomas upper right arm for >1 month and thrombocytopenia for was responsible for platelet trapping, which, by abnormally 1 day was admitted to Hunan Provincial People's Hospital proliferating endothelium within the hemangioma, can result (Changsha, China) and a series of treatments, including in the activation of platelets with secondary activation of γ-globulin impact, heparin anticoagulation, platelet transfu- coagulation cascades, eventually leading to consumption of sion, supplement of cryoprecipitate and fibrinogen following various clotting factors (7). An immunohistochemical study heparinization and inhabitation of vascular endothelial cell using monoclonal antibody against CD61, a marker of plate- proliferation by propranolol, were performed. At 2 months lets, and isotope studies using 111indium-labeled platelets after the initial admission to the hospital, surgery was and 51Cr-labeled platelets support the possible role of platelet conducted and the hemangioma was removed through trapping in the development of KMS (8). Patients with KMS pipeline arteriosclerosis embolization when the patient was develop local tumor intravascular coagulation, which leads hospitalized again with symptoms of vomiting and atrophy to severe thrombocytopenia, coagulation disorders, anemia accompanied by HC. The level of blood calcium reduced to and systemic inflammatory responses (9). Clinical manifesta- normal following surgery. Cases of KMS combined with HC tions of KMS, including local or systemic subcutaneous and are extremely rare and the most effective way to treat such visceral bleeding, high flow heart failure and red or purple cases is surgical resection of the hemangioma. vascular tumors, could be combined with laboratory blood tests, imaging or pathological examinations for diagnosis of Introduction this disease (10). The key to ensure effective treatment of KMS is to remove Congenital vascular lumps were previously generally referred or shrink the vascular tumors. Improving the condition of to as hemangioma; however, these vascular abnormalities disseminated intravascular coagulation and thrombocytopenia were classified in detail in 1982 by Mulliken and Glowacki in combined with glucocorticoid therapy remains the first‑line Harvard University (1). Based on different biological charac- treatment course of KMS (11). For patients who are insensitive teristics of endothelial cells and histopathological features, as to glucocorticoid therapy, other therapeutic methods, including well as clinical manifestations, vascular diseases are divided interferon therapy, β‑blockers, chemical treatment, anti‑platelet into two distinct categories: Hemangiomas and vascular drugs, radiotherapy, supportive care or combination therapy of glucocorticoids, could be given as alternative treatments. The International Association for the Study of Vascular Anomalies (ISSVA) classification method for vascular diseases may also be adapted to KMS, and the majority of pathological types Correspondence to: Professor Xiangling He, Department of of vascular tumors associated with KMS are acquired tufted Hematology and Oncology of Children's Medical Center, Hunan Provincial People's Hospital, 61 JieFang West Road, Changsha, angioma (ATA) or Kaposi hemangioendothelioma (KHE) (12). Hunan 410005, P.R. China KHE, possessing features of hemangioma and Kasposi's E-mail: [email protected] sarcoma, is a kind of locally aggressive or borderline vascular tumor, whereas ATA is considered as a benign vascular Key words: Kasabach-Merritt syndrome, hemangioma, tumor (13). thrombocytopenia, hypercalcemia Hypercalcemia (HC) refers to abnormal serum calcium increment and occurs when the amount of calcium absorbed into extracellular fluid (predominantly through intestine and bone) is much more than that discharged through intestinum ZOU et al: KASABACH‑MERRITT SYNDROME WITH HYPERCALCEMIA 6165 crassum and kidney (14). Clinical manifestations of HC vary range, 6‑14 fl). The coagulation function test indicated that greatly from asymptomatic phenotypes to hypercalcemic the fibrin degradation product was >100 µg/ml (normal range, crisis, and the latter is life-threatening (13,15). As the calcium 0‑5 µg/ml) and D‑dimer (DDI) was >10 mg/ml (normal range, balance is the combined effect of regulation through para- 0‑0.55 mg/l), with a prothrombin time of 14.3 sec (normal thyroid hormone (PTH), calcitonin and active vitamin D3 range, 10.0-15.5 sec), prothrombin time activity of 85.6% [1,25‑(OH)2D3] upon various organs, including intestine, (normal range, 80-140%), prothrombin time-international kidney and bones, abnormalities in any segments of this normalized ratio of 1.246 (normal range, 0.8‑1.5), fibrinogen regulatory process may lead to calcium metabolic distur- of 0.482 g/l (normal range, 2‑4 g/l), activated partial throm- bance (16). Typical causes of HC include hyperparathyroidism boplastin time of 43.3 sec (normal range, 24-38.6 sec), (primary, secondary or atopic), vitamin D metabolic disorder, thrombin time of 25.4 sec (normal range, 14-21 sec) and PTH-related protein secretion from tumors and many other antithrombin-III of 63.0% (normal range, 82-132%). Liver pathogenesis, including sarcoidosis, granulomatous, milk function examination demonstrated that alanine aminotrans- alkaline syndrome and adrenal insufficiency (17). ferase and aspartate aminotransferase were normal with The most common reason of HC syndrome is primary total bilirubin, direct bilirubin and indirect bilirubin levels hyperparathyroidism or malignancy-associated HC (18), of 145.9, 13.2 and 132.70 µmol/l, respectively. Bone marrow whereas cases of KMS combined with HC are extremely rare cytology results revealed that the patient developed prolifera- and have not been reported previously. The present report tive anemia bone marrow, while laboratory tests presented described the diagnosis and treatment of an infant with KMS normal antiplatelet antibodies. General lung CT scans and combined with HC. enhanced CT scanning indicated diffuse lesions in both lungs, therefore, the patient was suspected to have bronchial Case report pneumonia or pulmonary hemorrhage. As illustrated in Fig. 2, the results from MRI scans of the infant's upper arm Patient data. In September 2015, a 35-day-old male infant with combined with enhanced MRI, magnetic sensitive and MRV swelling on the upper right arm for >1 month and thrombocy- demonstrated occupancy lesions in the right armpit, upper topenia for 1 day was admitted to Hunan Provincial People's right arm as well as the right elbow, which were potential Hospital (Changsha, China) for the first time. The infant devel- hemangiomas. The result of blood gas analysis indicated that oped a dark purple lump in his upper right arm with a size of the pH value was 7.46, which was slightly higher than the 15x4x8 cm since birth. The guardians (parents of the patient) normal range (7.35-7.45). provided written informed consent for the publication of the clinical information of the patient. Treatment course. In line with all the examinations, the infant was diagnosed as KMS with manifestations of giant heman- Routine clinical examination. Following hospitalization, gioma and thrombocytopenia (19). The patient also developed a series of routine examinations, including blood routine severe anemia with hyperbilirubinemia and disseminated intra- examination, liver function test, marrow cytology inspec- vascular coagulation. A series of treatments, including heparin tion and antiplatelet antibody test, were performed. General anticoagulant, platelet transfusions as well as heparinized lung computed tomography (CT) scans and enhanced CT cryoprecipitate and fibrinogen supplement were implemented. scanning were also undertaken. In addition, magnetic reso- Methylprednisolone sodium succinate (China National nance imaging (MRI) scans of the infant's upper right arm Pharmaceutical Group Corportation, Beijing, China) injection followed by enhanced MRI and magnetic sensitive as well was also conducted, with a dose of 15 mg/kg per day for 3 days as magnetic resonance venography (MRV) were conducted. followed by a gradual reduction of dosage to 2 mg/kg per day Intracranial CT and abdominal ultrasonography were also for 9 days and sustained for a further 2 months. γ-globulin undertaken when the infant was hospitalized again at antiplatelet antibodies (Nanyue Biological Pharmaceutical Co., 90 days old. Ltd., Hengyang, China) were administered once. Propranolol (Jiangsu Yabang Epson Pharmaceutical Co. Ltd., Changzhou, General condition. The patient was born full-term, with China) was also prescribed (0.5 mg/kg per day
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