An 8-week-old male infant with cutaneous mass and consumption coagulopathy

Prepared by................... Pimlak Charoenkwan, M.D.
Chulabhorn Pruksachatkunakorn, M.D.
CC : Rreferred from a community hospital with hemangioma of left leg and thrombocytopenia
PI : The patient has had redness and swelling of left leg since birth. He was admitted to a community hospital where the lesion was suspected as a result of birth injury. He was given intravenous antibiotics for 2 weeks to treat as cellulitis. Initially the swelling partially resolved but later worsened. PTT was prolonged at that time. Platelet count was 13,000. He was given vitamin K and cloxacillin. Because of the persistent redness and swelling, a diagnosis of hemangioma was suspected and he was transferred to Maharaj Nakhorn Chiang Mai Hospital.
PH : The patient was born at term. Additional history is not available.
Significant PE

Vital signs: T 37.4C PR 130/min RR 36/min BP 87/40 mmHg
Weight 6,150g Length 55cm OFC 39.5 cm
GA: A male infant, active
HEENT: not pale, no icteric sclera
Lymph nodes: not palpable
Heart: regular, normal S1,S2, no murmur
Lungs: clear, no adventitious sounds
Abdomen: liver 1 cm below right costal margin
Ext: left hip in abduction position, decreased movement of left leg
Skin: large, well-circumscribed violaceous indurated plaques, fixed to overlying and underlying skin and soft tissue below left knee (Figure 1,2), two ill-defined bluish firm mass, 1.5 cm in diameter at left inguinal area

Laboratory investigations:
CBC: Hb 8.4 g%, Hct 25%, WBC 10,400/cu.mm.(N52%, L24%, M24%)
Plt 13,000/cu.mm.
PBS: normochromic normocytic, a few fragmented RBC, decreased number of platelets
PT>200 (control 11.2), INR>10, PTT>200 (control 27.5)
Fibrinogen: 57 mg% (normal 220-320 mg%)
D-dimer 1 ng/mL(normal< 0.5 ng/mL)
U/S liver: no hemangioma
Skin biopsy (performed prior to referral): increased vascular channels on superficial dermis

Problem list:
1. Enlarging cutaneous mass since birth (differential diagnosis of hemangioma or vascular tumor)
2. Consumption coagulopathy

Diagnosis:
Kasabach-Merritt syndrome
Treatment:
1. Specific treatment (given stepwise according to the response):
-prednisolone 4 mg/kg/day
-pulse methylprednisolone
-chemotherapy: vincristine
2. Supportive treatment: Cryoprecipitate and platelet transfusions
3. Monitor clinical progression, and close observance for DIC: hematological parameters (Hb, Plt count, fibrinogen, FDP, D-dimer)
Kasabach-Merritt syndrome
This syndrome is a combination of a rapidly enlarging vascular anomaly, thrombocytopenia, microangiopathic hemolytic anemia, and an acute or chronic consumption coagulopathy. The vascular lesion has been presumed to be a hemangioma but alternatively may be a tufted angioma or a kaposiform hemangioendothelioma with lymphatic-like vessels. The clinical manifestations are usually evident during early infancy, but the onset occasionally is later. The associated platelet defect may lead to precipitous hemorrhage accompanied by ecchymosis, petechiae and a rapid increase in size of the vascular lesion. Severe anemia as a result of hemorrhage or microangiopathic hemolysis may ensue. The platelet count is depressed, but the bone marrow contains increased numbers of normal or immature megakaryocytes. The thrombocytopenia has been attributed to sequestration or increased destruction of platelets within the hemangioma. Hypofibrinogenemia and decreased levels of consumable clotting factors are relatively common. Treatment includes management of thrombocytopenia, anemia and consumptive coagulopathy by administering platelets and by transfusion of red blood cells and fresh frozen plasma. Heparinization is controversial but has benefited some patients when combined with transfusions, Arteriovenous shunts in large lesions may produce high-output heart failure requiring digitalization. Treatment of these lesions includes systemic steroids, embolization, radiation therapy, aminocaproic acid (inhibit fibrinolysis), cyclophosphamide, pentoxifylline, or recombinant IFN-alpha, which may inhibit proliferation of endothelial and smooth muscle cells. The mortality rate is 20-30%. (from Nelson Textbook of Pediatrics 16th ed. p.1977)
Suggested readings:
Hall W. Kasabach-Merritt syndrome: pathogenesis and management. Br J Haematol 2001;112:851-62.
Powell J. Update on hemangiomas and vascular malformations. Curr Opin Pediatr 1999;11:457-63.
Diagnosis : Kasabach-Merritt syndrome

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