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Thursday, October 25, 2007

Kasabach-Merritt Syndrome

I came across this very intresting case by By: patricia burrows, Radiologist, Children's Hospital Boston, Massachusetts, USA

these are great MRI's of this Syndrome

Here is another good case

Thanks mypacsnet

General Discussion
Hemangioma-Thrombocytopenia Syndrome (also known as Kasabach-Merritt Syndrome) is a rare disorder characterized by an abnormal blood condition in which the low number of blood platelets causes bleeding (thrombocytopenia). The thrombocytopenia is found in association with a benign tumor consisting of large, blood-filled spaces (cavernous hemangioma). The exact cause of this disorder is not known.



Symptoms

Hemangioma-Thrombocytopenia Syndrome is a rare disorder that is typically characterized by a benign (non-cancerous) tumor consisting of large, blood- filled spaces (cavernous hemangioma). This tumor occurs along with a blood condition (thrombocytopenia) characterized by an abnormally low number of blood platelets which can cause excessive bleeding.Excessive bleeding (hemorrhaging) beneath the skin (purpura) typically develops within the first six weeks of life, but may appear later in childhood as the hemangioma increases in size. Usually there is only one hemangioma which is found on the neck, arms, legs, or trunk of the body. Less common are tumors of the internal organs which may be found on the tongue, thorax, spleen, liver, gastrointestinal tract or bones. Hemangiomas are rarely found internally and on the skin of the same patient.Thrombocytopenia may occur within the first month of life in association with a hemangioma of the outer layer of the placenta (placental chorioangioma) or large hemangiomas of the skin.Hemangiomas may become filled with blood (engorged) before a bleeding spell occurs. The cause of the bleeding is not certain, but it can be triggered by trauma.A decrease in the level of the oxygen carrying component of blood cells (hemoglobin), red blood cells (erythrocytes), and/or proteins in the blood that are part of the clotting process (prothrombin and fibrinogen) may also occur. However, fibrinogen deficiency usually affects older children and adults.CausesThe exact cause of Hemangioma-Thrombocytopenia Syndrome is not known. It is thought that the thrombocytopenia may be a result of platelet destruction which occurs in relationship to growth of the hemangioma. There is no evidence that the syndrome is hereditary.Affected PopulationsHemangioma-Thrombocytopenia Syndrome is a very rare disorder that affects males and females in equal numbers. Approximately one in every five hundred cases of people with hemangiomas have associated thrombocytopenia.Courtesy of NORD website: http://rarediseases.org/



11 month 22 day old male: 11-month-old with history of Kasabach-Merritt syndrome and left shoulder/arm hemangioma, presenting with thrombocytopenia. The patient is a full term spontaneous vaginal delivery with no complications during pregnancy. At six weeks of age at a well baby checkup, the patient was noted to have two small bruises on the left arm and deltoid region. At three months the patient's mother noticed increased area of bruising as well as two more bruises on the left shoulder area. Diagnosis was hemangioma which would resolve with time. A left shoulder incisional biopsy was consistent with hemangioma. In September of 1994 he was placed on Prednisone, 4 milligrams per kilograms per day. Prednisone initially resulted in improvement but then the hemangioma increased in size. The patient was first given platelets in September of 1994 for a platelet count of 22K. Mother estimates some 30 platelet transfusions since then, the last one being in December of 1994. The patient underwent three sessions of radiation treatment in December of 1994, and on December 22, 1994 the patient was admitted with the diagnosis of rectal prolapse, which was reduced. Cultures grew out Klebsiella and the patient was treated with Tobramycin and Timentin. The patient improved and was discharged. Interferon was begun in October of 1994, present dose is 0.3 milliliters subcutaneously q.d. Two urine samples of FgF showed on January 30, 1995 44,000, and on February 27th 32,000. Three days prior to admission the patient showed increased agitation. Hemangioma became darker and harder, and most recent complete blood count as of April 3, 1995 was a red blood cells of 4.53, hematocrit of 23.2, and platelet count of 5. The white blood cell count had a differential of 15 polys, 7 bands, 61 lymphs, 5 monos, 2 eosinophils. There has been no history of significant bleeding.

more info

Case study of IAC involvement.

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MRI Nueroarm Video