Leukemic cells may gather in the spleen and cause it to swell; this can have the side effect of making the person feel full even when he or she has not eaten much.Hairy cell leukemia is commonly diagnosed after a routine blood count shows unexpectedly low numbers of one or more kinds of normal blood cells, or after unexplained bruises or recurrent infections in an otherwise apparently healthy patient.Platelet function may be somewhat impaired in HCL patients, although this does not appear to have any significant practical effect. It may result in somewhat more mild bruises than would otherwise be expected for a given platelet count or a mildly increased bleeding time for a minor cut, likely the result of producing slightly abnormal platelets in the overstressed bone marrow tissue. Patients with a high tumor burden may also have somewhat reduced levels of cholesterol, especially in patients with an enlarged spleen. Cholesterol levels return to more normal values with successful treatment of HCL. Cause
As with many cancers, the cause of HCL is unknown. Exposure to tobacco smoke, ionizing radiation, or industrial chemicals (with the possible exception of diesel) does not appear to increase the risk of developing it.
This would include:
Sequencing of SRCAP exons 31–34 in all suspected cases
Complete assessments of auditory and visual systems
Renal and urinary tract ultrasound
Orthopedic assessment of hip dysplasia and clavicle abnormalities
Neurologic assessment if there is a suspicion of seizures
Dental hygiene to prevent cavities and to monitor for malocclusion
Evaluation for growth hormone deficiency at baseline, to be repeated if loss of growth velocity occurs
Monitoring of bone age and pubertal timing in case of precocious puberty
Psychoeducational assessments corrected for deficiencies in expressive language and sensory issues
Monitoring of behavioral disturbances and provision of early intervention
Counseling for families regarding recurrence risk and the offspring of individuals with FHSSpecial education programs and vocational training to address developmental disabilities are highly recommended, as well as communication rehabilitation with sign language or alternative means of communication. Behavior management strategies could also include referrals to behavior specialists or psychologists for help. For those concerned, genetic counseling can be sought for issues related to testing of at-risk relatives. History
The first identified instances occurred in 1973 at the Boston Floating Hospital, and in 1975 at Harbor General Hospital in Torrance, California. The name Floating–Harbor syndrome was coined by Robinson et al. in 1988. Since then approximately 40 more cases have been described.The first case recorded was that of a 5-year-old boy. In a 32-year follow up done in 2006, the patient was in good overall health, had never been hospitalized, and had been employed for the past 15 years. His mother stated he had a very good memory, was gregarious, had a temper, and at times was stubborn.