Table 4

Tier 3 treatment options

TreatmentReferenceDoseResponse rate (%)Selected toxicities
ATRA 86 10 mg orally 3 times per day 29 Retinoic acid syndrome, flu-like symptoms, musculoskeletal pain, nausea/vomiting, peripheral neuropathy 
Autologous HSCT 87 Cyclophosphamide 50 mg/kg IV once per day × 4 days (conditioning) 43 Neutropenic fever, infection 
Colchicine 88 1.2 g orally once per day 21 Agranulocytosis, neuritis, diarrhea, nausea/vomiting 
Interferon α 89-92 Various 0-36 Neutropenia, fever, influenza-like symptoms, hepatotoxicity 
Plasma exchange 93-95 One plasma volume exchange once per day × 1 to 8 days 29-80 Hypocalcemia, anaphylactoid reactions 
Protein A immunoadsorption 96 Average of 6 treatments (0.25 to 2.0 L plasma per treatment) over 2 to 3 weeks 21 Hypersensitivity reactions, pain, nausea/vomiting 
Cardiopulmonary complications 
Vitamin C 97-100 2 g orally once per day 0-82 Dsypepsia, nausea/vomiting 
TreatmentReferenceDoseResponse rate (%)Selected toxicities
ATRA 86 10 mg orally 3 times per day 29 Retinoic acid syndrome, flu-like symptoms, musculoskeletal pain, nausea/vomiting, peripheral neuropathy 
Autologous HSCT 87 Cyclophosphamide 50 mg/kg IV once per day × 4 days (conditioning) 43 Neutropenic fever, infection 
Colchicine 88 1.2 g orally once per day 21 Agranulocytosis, neuritis, diarrhea, nausea/vomiting 
Interferon α 89-92 Various 0-36 Neutropenia, fever, influenza-like symptoms, hepatotoxicity 
Plasma exchange 93-95 One plasma volume exchange once per day × 1 to 8 days 29-80 Hypocalcemia, anaphylactoid reactions 
Protein A immunoadsorption 96 Average of 6 treatments (0.25 to 2.0 L plasma per treatment) over 2 to 3 weeks 21 Hypersensitivity reactions, pain, nausea/vomiting 
Cardiopulmonary complications 
Vitamin C 97-100 2 g orally once per day 0-82 Dsypepsia, nausea/vomiting 

ATRA, all-trans retinoic acid; HSCT, hematopoietic stem cell transplant

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