A probable diagnosis is the same but with one secondary accessory criterion. Classification
2003 nomenclature
IA - Fas
IB - Fas ligand
IIA - Caspase 10
IIB  - Caspase 8
III - unknown
IV - Neuroblastoma RAS viral oncogene homologRevised nomenclature (2010)
ALPS-FAS: Fas. Germline FAS mutations. 70% of patients. Autosomal dominant. Dominant negative and haploinsufficient mutations described. ALPS-sFAS: Fas. Somatic FAS mutations in DNT compartment. 10% of patients
ALPS-FASL: Fas ligand. Germline FASL mutations. 3 reported cases
ALPS-CASP10: Caspase 10. Germline CASP10 mutation. 2% of patients
ALPS-U: Undefined. 20% of patients
CEDS: Caspase 8 deficiency state. No longer considered a subtype of ALPS but distinct disorder
RALD: NRAS, KRAS. Somatic mutations in NRAS and KRAS in lymphocyte compartment. No longer considered a subtype of ALPS but distinct disease

Treatment
Treatment is most commonly directed at autoimmune disease and may be needed to treat bulky lymphoproliferation. First line therapies include corticosteroids (very active but toxic with chronic use), and IVIgG, which are not as effective as in other immune cytopenia syndromes.Second line therapies include: mycophenolate mofetil (cellcept) which inactivates inosine monophosphate, most studied in clinical trials with responses varying (relapse, resolution, partial response). It does not affect lymphoproliferation or reduce DNTs, with no drug-drug interactions. This treatment is commonly used agent in patients who require chronic treatment based on tolerance and efficacy.
However, the evidence of this effect has been disputed, and these medications do have negative side effects. For patients with nerve root pain and acute radiculopathy, there is evidence that a single dose of steroids, such as dexamethasone, may provide pain relief. Epidural corticosteroid injection (ESI) is a procedure in which steroid medications are injected into the epidural space. The steroid medications reduce inflammation and thus decrease pain and improve function. ESI has long been used to both diagnose and treat back pain, although recent studies have shown a lack of efficacy in treating low back pain. Surgery
Surgery for back pain is typically used as a last resort, when serious neurological deficit is evident. A 2009 systematic review of back surgery studies found that, for certain diagnoses, surgery is moderately better than other common treatments, but the benefits of surgery often decline in the long term.Surgery may sometimes be appropriate for people with severe myelopathy or cauda equina syndrome. Causes of neurological deficits can include spinal disc herniation, spinal stenosis, degenerative disc disease, tumor, infection, and spinal hematomas, all of which can impinge on the nerve roots around the spinal cord. There are multiple surgical options to treat back pain, and these options vary depending on the cause of the pain. When a herniated disc is compressing the nerve roots, hemi- or partial- laminectomy or discectomy may be performed, in which the material compressing on the nerve is removed. A mutli-level laminectomy can be done to widen the spinal canal in the case of spinal stenosis.