Patients >60 years or less medically fit are given cyclophosphamide, doxorubicin, vincristine, and prednisone while patients ≤60 years old are given CHOP plus etoposide or one of various other intensive chemotherapy regiments. The intensive chemotherapy regimens give 5 year overall survival rates of 70–93%. The role of radiation therapy for ALK-positive ALCL is unclear but has been used for patients who cannot tolerate or do not achieve complete responses to the drug regimens and to patients with organ-threatening or life-threatening tumorous infiltrates. The role of autologous or less preferably allogenic hematopoietic stem cell transplantation (transplantation using the individuals own bone marrow, i.e. autologous, or a donors, i.e. allogenic) after achieving a complete remission following induction therapy is also unclear. Individuals with relapsed or refractory disease are treated with brentuximab vedotin if they did not receive the drug previously or had not received it in the previous 6 months. A small study reported overall response rates, complete response rates, and disease-free survival rates at 24 months of 63%, 45%, and 54%, respectively, using this regimen. Those who attain a complete response on this drug and can tolerate it are than treated with bone marrow transplantation. Finally, patients who fail or relapse on these treatments are given salvage therapy regimens that have been used for relapsed or refractory aggressive B cell malignancies such as GDP (i.e.
Oculofaciocardiodental syndrome is a rare X-linked dominant genetic disorder. Presentation
The incidence of this condition is less than 1 per million. It is primarily only found in females. Its highly rare in males, but some males were born with it. Teeth with large roots (radiculomegaly), heart defects and small eyes (microphthalmia) are the characteristic triad found in this syndrome. Typical features of the condition include:
Face
Deep set eyes
Broad nasal tip divided by a cleft
Eyes
Microphthalmia (small eyes)
Early cataracts
Glaucoma
Teeth
Radiculomegaly (teeth with very large roots)
Delayed loss of primary teeth
Missing (oligodontia) or abnormally small teeth
Misaligned teeth
Defective tooth enamel
Heart defects
Atrial and/or ventricular defects
Mitral valve prolapse
Mild intellectual disability and conductive or sensorineural hearing loss may occur. Genetics
This condition is caused by lesions in the BCOR gene located on the short arm of the X chromosome (Xp11.4). This protein encodes the BCL6 corepressor, but little is currently known about its function. The inheritance is X-linked dominant.A genetically related disorder is Lenz microphthalmia syndrome. Diagnosis
Diagnosis can be confirmed through DNA testing. Treatment
History
The first features of this syndrome noted were the abnormal teeth, which were described by Hayward in 1980. == References ==