Page 1981 - Hematology_ Basic Principles and Practice ( PDFDrive )

Chapter 115 Transfusion of Plasma and Plasma Derivatives 1755

(murine monoclonal antibody to CD3 antigen of human T cells). in association with monoclonal Igs. One placebo-controlled trial
Consequently, current consensus renal transplant guidelines indicate demonstrated that IVIg may improve short-term morbidity, but the
IVIg is a useful treatment modality for desensitization of patients remainder of the available evidence is mixed. The use of IVIg for this
with HLA antibodies and in patients with acute rejection. condition has recently fallen out of favor with current consensus
Randomized trials have not yet been performed for ABO incom- groups.
patible kidney transplants, heart, liver, or lung transplants. Moreover,
there is a paucity of data for transplant outcomes, and current studies
have small numbers without data on donor specific antibody levels. Inclusion Body Myositis
Current guidelines assert that there is insufficient evidence to make
a recommendation for or against the routine use of IVIg for desen- Inclusion body myositis is an inflammatory myopathy result-
sitization in these transplants. ing in chronic muscular weakness. Randomized trials using IVIg
appear to result in short-term improvement in strength scores and
improved swallowing in patients with inclusion body myositis,
Aplastic Anemia Secondary to Parvovirus and are equivalent to treatment with glucocorticosteroids in one
small clinical study. The use of IVIg for this condition has also
Parvovirus B19 infection can result in severe anemia and reticulocy- recently fallen out of favor with current consensus groups because
topenia, especially in immunocompromised individuals or individuals of the lack of known sustained benefit for patients with this
with sickle cell disease or thalassemia, and the use of IVIg is consid- condition.
ered first-line therapy in the treatment of these patients (typical dose
0.5 g/kg weekly for 4 weeks).
Lambert-Eaton Myasthenic Syndrome
Chronic Inflammatory Demyelinating Lambert-Eaton myasthenic syndrome results from antibodies to the
Polyradiculoneuropathy neuromuscular junction, leading to autonomic dysfunction. One
randomized control trial reveals that IVIg significantly improves
CIDP is a chronic disorder resulting in demyelination of peripheral generalized central and peripheral muscle strength and decreases
nerves that result in weakness and sensory changes. Equivalent out- serum calcium channel antibody titers. A total dose of 2.0 g/kg given
comes have been observed in the treatment of CIDP with IVIg over 2 to 5 days is a recommended initial treatment.
(reported dose 400 mg/kg/day for 5 days, once each month, or
1 g/kg/day for 2 days, once each month), TPE, or glucocorticoste-
roids. The decision as to which treatment to use is made on an Multifocal Motor Neuropathy
individual basis balancing the risks and benefits of each treatment
modality. Multifocal motor neuropathy is a chronic progressive disorder result-
ing in primarily hand weakness. IVIg is now considered a first-line
treatment for this condition, and improved strength can be seen at a
Dermatomyositis dose of 2.0 g/kg over 2 to 5 days.

Dermatomyositis is a chronic inflammatory disorder that results in
progressive weakness and rash. IVIg (typical dose 2.0 g/kg per month Multiple Sclerosis
administered over 2–5 days) results in improved muscle strength and
neuromuscular symptoms. Multiple sclerosis is a chronic progressive or relapsing and remitting
disorder characterized by brain white mater demyelination. There are
Guillain-Barré Syndrome (Acute Inflammatory two published metaanalyses, and several randomized controlled clini-
cal trials in patients with relapsing-remitting multiple sclerosis using
Demyelinating Polyneuropathy) a wide range of IVIg doses that demonstrate the success of IVIg in
reducing the number of exacerbations and disability in patients with
Guillain-Barré syndrome is an acute demyelinating peripheral neu- relapsing-remitting multiple sclerosis in comparison with placebo.
ropathy affecting both motor and sensory nerves. IVIg (typical dose However, no studies to date have compared IVIg with standard
400 mg/kg/day for 5 days or 1.0 g/kg/day for 2 days or 2.0 g/kg as therapies, and one clinical trial (PRIVIG trial) has raised doubt that
a single dose) is likely equivalent to TPE in improving disability and IVIg is effective as a routine treatment. Consequently, IVIg is con-
shortening the time to improvement. sidered a viable second-line option for those patients who fail, decline,
or are unable to tolerate standard immunomodulatory therapies such
Hypogammaglobulinemia Associated as β-interferon and glatiramer acetate.
With Multiple Myeloma
Myasthenia Gravis
Multiple myeloma is a monoclonal B-cell (plasma cell) disorder with
clinical symptoms arising as a result of plasma cell infiltration of the Myasthenia gravis is a chronic neurologic autoimmune disorder
bone marrow, monoclonal Ig in the blood and urine, and immuno- characterized by weakness and fatigue upon repetitive skeletal muscle
suppression. IVIg has shown to be beneficial in preventing serious use, which improves with rest. IVIg has been used successfully as a
infections in plateau-phase multiple myeloma or other hematologic short-term measure for acute severe exacerbations of myasthenia
malignancy, where the patients have hypogammaglobinemia, at doses gravis at a dose of 2.0 g/kg given over 2 to 5 days, and appears
of 0.4 g/kg every 4 weeks, with subsequent dosing adjusted based on comparable with TPE. A definitive randomized control trial compar-
trough levels. ing the two treatment modalities has not been done, however.

IgM Paraproteinemic Demyelinating Neuropathy Neonatal Alloimmune Thrombocytopenia
Paraproteinemic demyelinating neuropathy is a chronic disorder NAIT is a rare condition that results from maternal platelet alloan-
resulting in decreased sensory and motor function, similar to CIDP, tibodies against the fetal/neonatal platelets resulting in neonatal/fetal