Dosage and administration
Inhaled
In the U.S., iloprost is inhaled specifically using the I-Neb AAD or Prodose AAD delivery systems. In Europe iloprost has been approved for use with two compressed air nebulizers with AAD delivery systems (Halolite and Prodose) as well as with two ultrasonic nebulizers Ventaneb  and I-Neb. Ventavis is supplied in 1 mL single-use glass ampules containing either 10 μg/mL or 20 μg/mL. The 20 μg/mL concentration is intended for patients who are maintained at the 5 μg dose and who have repeatedly experienced extended treatment times which could result in incomplete dosing. Transitioning patients to the 20 μg/mL concentration using the I-neb AAD System will decrease treatment times to help maintain patient compliance.The approved dosing regimen for iloprost is 6 to 9 times daily (no more than every 2 hours) during waking hours, according to individual need and tolerability. The significant clinical effects observed in the pivotal study of patients with PAH were achieved with a median dose of 30 μg per day (range: 12.5 to 45 μg delivered at the mouthpiece), corresponding to 6 daily inhalations of 5 μg. The majority of patients (> 80%) in the pivotal study used this median dose or a higher dose with an excellent treatment compliance after 12 weeks. The first inhaled dose of iloprost should be 2.5 μg (as delivered at the mouthpiece). If this dose is well tolerated, dosing should be increased to 5 μg and maintained at that dose.
Until this point, only a few genomic imbalances had been found in the hairy cells, such as trisomy 5 had been found. The expression of genes is also dysregulated in a complex and specific pattern. The cells underexpress 3p24, 3p21, 3q13.3-q22, 4p16, 11q23, 14q22-q24, 15q21-q22, 15q24-q25, and 17q22-q24 and overexpress 13q31 and Xq13.3-q21. It has not yet been demonstrated that any of these changes have any practical significance to the patient. Diagnosis
The diagnosis of HCL may be suggested by abnormal results on a complete blood count (CBC), but additional testing is necessary to confirm the diagnosis. A CBC normally shows low counts for white blood cells, red blood cells, and platelets in HCL patients. However, if large numbers of hairy cells are in the blood stream, then normal or even high lymphocyte counts may be found. On physical examination, 80–90% of patients have an enlarged spleen, which can be massive. This is less likely among patients who are diagnosed at an early stage. Peripheral lymphadenopathy (enlarged lymph nodes) is uncommon (less than 5% of patients), but abdominal lymphadenopathy is a relatively common finding on computed tomography scans. The most important laboratory finding is the presence of hairy cells in the bloodstream. Hairy cells are abnormal white blood cells with hair-like projections of cytoplasm; they can be seen by examining a blood smear or bone marrow biopsy specimen. The blood film examination is done by staining the blood cells with Wrights stain and looking at them under a microscope.