Lymphoplasmacytic lymphoma medical therapy: Difference between revisions

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==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
There are several different options for treating Waldenström macroglobulinemia depending on stage of the disease:<ref name="Tx">Lymphoplasmacytic lymphoma. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/lymphoplasmacytic-lymphoma/?region=ab Accessed on November 6 2015 </ref>
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
===Disease Name===


* '''1 Stage 1 - Name of stage'''
====Asymptomatic/Smoldering Waldenström's Macroglobulinemia====
** 1.1 '''Specific Organ system involved 1'''
There is no treatment for asymptomatic Waldenström macroglobulinemia. Asymptomatic waldenström's macroglobulinemia can be monitored every 3-6 months.<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref> Active surveillance includes monitoring of the following laboratory parameters:
*** 1.1.1 '''Adult'''
*Complete blood count ([[Complete blood count|CBC]]) with differential
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
*Complete metabolic panel ([[CMP-N-acetylneuraminate monooxygenase|CMP]])
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
*Immunoglobulin levels in the serum (quantitative)
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
*Serum protein electrophoresis
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*** 1.1.2 '''Pediatric'''
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


* 2 '''Stage 2 - Name of stage'''
====Symptomatic Waldenström's Macroglobulinemia====
** 2.1 '''Specific Organ system involved 1 '''
Symptomatic patients with waldenström macroglobulinemia are started on chemotherapy depending on the stage.<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>
**: '''Note (1):'''
 
**: '''Note (2)''':  
*Initial stage of waldenström's macroglobulinemia associated with:
**: '''Note (3):'''
:*[[Neuropathy]]
*** 2.1.1 '''Adult'''
:*[[Anemia]] or [[cytopenias]]
**** Parenteral regimen
:*Low-volume nodal involvement
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
:*Asymptomatic [[splenomegaly]]
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
 
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
*Late stage of Waldenström's macroglobulinemia associated with:
**** Oral regimen
:*[[Adenopathy]]
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
:*Symptomatic [[splenomegaly]]
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
:*[[Cytopenia|Cytopenias]]
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
:*[[Hyperviscosity syndrome]]
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
:*[[Neuropathy]]
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
:*Constitutional symptoms
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
 
*** 2.1.2 '''Pediatric'''
{| style="border: 0px; font-size: 90%; margin: 3px; width: 800px"
**** Parenteral regimen
| valign="top" |
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
|+
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Treatment Regimen<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
}}
**** Oral regimen
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Drugs}}
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Side effects}}
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
|-
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
'''[[CHOP-R regimen]]'''  
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
| style="padding: 5px 5px; background: #F5F5F5;" |
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
*[[Cyclophosphamide]]
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
*[[Doxorubicin]]
**: '''Note (1):'''
*[[Vincristine]]
**: '''Note (2)''':  
*[[Prednisone]]
**: '''Note (3):'''
*[[Rituximab]]
*** 2.2.1 '''Adult'''
| style="padding: 5px 5px; background: #F5F5F5;" |
**** Parenteral regimen
*[[Nausea]]
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*[[Alopecia]]
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
*[[Granulocytopenia]]
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
*[[Cardiotoxicity]]
**** Oral regimen
*[[Mucositis]]
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
|-
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
'''[[Ibrutinib]]'''
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
| style="padding: 5px 5px; background: #F5F5F5;" |
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*[[Ibrutinib]]
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
| style="padding: 5px 5px; background: #F5F5F5;" |
*** 2.2.2 '''Pediatric'''
*[[Fatigue]]
**** Parenteral regimen
*[[Cytopenia]]
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
*[[Bleeding]]
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
*[[Atrial fibrillation]]
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
*[[Opportunistic infection]]
**** Oral regimen
|-
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
'''[[Rituximab]]'''
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
| style="padding: 5px 5px; background: #F5F5F5;" |
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
*[[Rituximab]]
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
| style="padding: 5px 5px; background: #F5F5F5;" |
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
*Infusion related reaction
*[[Hepatitis B]] reaction
*Progressive multi-focal leukoencephaloptahy
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''FR regimen'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Fludarabine]]
*[[Rituximab]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Neutropenia]] (63%)
*[[Thrombocytopenia]]
*[[Pneumonia]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''BDR regimen'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Bortezomib]]
*[[Dexamethasone]]
*[[Rituximab]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Peripheral neuropathy]] - reversible in 61% of patients
*[[Infections]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''DRC regimen'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Dexamethasone]]
*[[Rituximab]]
*[[Cyclophosphamide]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Neutropenia]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''CR regimen'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Cladribine]]
*[[Rituximab]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Anemia]]
*Neurological symptoms
*Symptomatic [[cryoglobulinemia]]
*[[Thrombocytopenia]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''IR regimen'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Ibrutinib]]
*[[Rituximab]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Anemia]]
*Neurological symptoms
*Symptomatic [[cryoglobulinemia]]
*[[Thrombocytopenia]]
*[[Atrial fibrillation]]
|-
|}
 
====Hyperviscosity syndrome====
*Waldenström macroglobulinemia complicated with [[hyperviscosity syndrome]] is a medical emergency and requires prompt treatment with plasmapheresis.<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>
*[[Plasmapheresis]] temporarily lowers [[IgM]] levels by removing some of the abnormal IgM from the blood, which makes blood thinner.
*Plasmapheresis is usually given until chemotherapy starts to work.
*Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.


==References==
==References==

Revision as of 18:04, 12 February 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

There are several different options for treating Waldenström macroglobulinemia depending on stage of the disease:[1]

Asymptomatic/Smoldering Waldenström's Macroglobulinemia

There is no treatment for asymptomatic Waldenström macroglobulinemia. Asymptomatic waldenström's macroglobulinemia can be monitored every 3-6 months.[2] Active surveillance includes monitoring of the following laboratory parameters:

  • Complete blood count (CBC) with differential
  • Complete metabolic panel (CMP)
  • Immunoglobulin levels in the serum (quantitative)
  • Serum protein electrophoresis

Symptomatic Waldenström's Macroglobulinemia

Symptomatic patients with waldenström macroglobulinemia are started on chemotherapy depending on the stage.[3]

  • Initial stage of waldenström's macroglobulinemia associated with:
  • Late stage of Waldenström's macroglobulinemia associated with:
Treatment Regimen[3]

Drugs Side effects

CHOP-R regimen

Ibrutinib

Rituximab

  • Infusion related reaction
  • Hepatitis B reaction
  • Progressive multi-focal leukoencephaloptahy

FR regimen

BDR regimen

DRC regimen

CR regimen

IR regimen

Hyperviscosity syndrome

  • Waldenström macroglobulinemia complicated with hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.[3]
  • Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes blood thinner.
  • Plasmapheresis is usually given until chemotherapy starts to work.
  • Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.

References

  1. ↑ Lymphoplasmacytic lymphoma. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/lymphoplasmacytic-lymphoma/?region=ab Accessed on November 6 2015
  2. ↑ Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
  3. ↑ 3.0 3.1 3.2 Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015

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