Osteosclerotic plasma cell myeloma: Difference between revisions

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{{short description|Cancer of plasma cells}}
{{short description|Cancer of plasma cells characterized by osteosclerosis}}
{{Distinguish|Malignant melanoma}}
{{Distinguish|Multiple myeloma}}
{{Infobox medical condition (new)
{{Infobox medical condition (new)
| name          = Multiple myeloma
| name          = Osteosclerotic plasma cell myeloma
| image        = Plasmacytoma_ultramini1.jpg
| image        = Plasmacytoma_ultramini1.jpg
| caption      = [[Micrograph]] of a [[plasmacytoma]], [[H&E stain]]
| caption      = [[Micrograph]] of a [[plasmacytoma]] with osteosclerotic changes, [[H&E stain]]
| synonym      = Plasma cell myeloma, myelomatosis, Kahler's disease, myeloma<ref>{{cite web |title=Myeloma Canada {{!}} What is Multiple Myeloma? |url=https://www.myelomacanada.ca/en/about-multiple-myeloma/what-is-myeloma |website=www.myelomacanada.ca |access-date=17 April 2020}}</ref>
| field        = [[Hematology]] and [[oncology]]
| field        = [[Hematology]] and [[oncology]]
| symptoms      = [[Bone pain]], fatigue<ref name=":5" />{{Rp|653}}
| symptoms      = [[Bone pain]], [[fatigue]], [[fractures]]
| complications = [[Amyloidosis]], [[kidney problems]], [[pathologic fracture|bone fractures]], [[hyperviscosity syndrome]], infections, [[anemia]]<ref name="NIH2016Pro" /><ref name=":5" />{{Rp|653}}
| complications = [[Amyloidosis]], [[kidney disease]], [[pathologic fractures]], [[anemia]], [[infections]], [[nerve damage]]
| onset        =  
| onset        = Gradual
| duration      = Long term<ref name="NIH2016Pro" />
| duration      = Long term
| causes        = Unknown<ref name="WCR2014Cp513" />
| causes        = Unknown
| risks        = [[Obesity]]<ref name="WCR2014Cp2" />
| risks        = [[Obesity]], [[genetic predisposition]]
| diagnosis    = Blood or urine tests, [[bone marrow biopsy]], [[medical imaging]]<ref name="Raab2009" />
| diagnosis    = [[Blood test]], [[urine test]], [[bone marrow biopsy]], [[medical imaging]]
| differential  =
| treatment    = [[Steroids]], [[chemotherapy]], [[stem cell transplantation]], [[bisphosphonates]], [[radiation therapy]]
| prevention    =
| prognosis    = Variable based on stage and treatment
| treatment    = [[glucocorticoid|Steroids]], [[chemotherapy]], [[thalidomide]], [[stem cell transplant]], [[bisphosphonates]], [[radiation therapy]]<ref name="NIH2016Pro" /><ref name="Raab2009" />
| frequency    = Rare
| medication    =
| prognosis    = [[Five-year survival rate]] 54% / life expectancy 6 years (USA)<ref name="SEER2016" />
| frequency    = 488,200 (affected during 2015)<ref name="GBD2015Pre" />
| deaths        = 101,100 (2015)<ref name="GBD2015De" />
| alt          =
}}
}}
A [[plasma cell]] [[neoplasm]] associated with [[osteosclerosis|osteosclerotic]] and fibrotic changes in the bone trabeculae.


==Lymph node changes==
'''Osteosclerotic plasma cell myeloma''' is a rare subtype of [[plasma cell neoplasm]] characterized by [[osteosclerosis]], a condition in which abnormal hardening occurs in the bones. This variant is distinct from other forms of [[multiple myeloma]] due to its association with [[bone formation]] abnormalities and fibrotic changes in the bone trabeculae.  
Often, the [[lymph node]]s show changes resembling the plasma cell variant of [[castleman disease]].  


==POEMS syndrome==
== Pathophysiology ==
It is often part of [[poems syndrome]] which is characterized by [[polyneuropathy]], [[organomegaly]], [[endocrinopathy]], [[monoclonal gammopathy]], and skin changes.
Osteosclerotic plasma cell myeloma involves the proliferation of abnormal [[plasma cells]] within the [[bone marrow]], which leads to the replacement of normal bone marrow elements. Unlike traditional [[osteolytic lesions]] seen in classic [[multiple myeloma]], this subtype is marked by [[osteosclerosis]], resulting in increased bone density. These changes are due to the interaction between cancerous plasma cells and [[osteoblast]]s, the cells responsible for bone formation.
 
The disease is also linked to excessive production of [[monoclonal gammopathy|monoclonal antibodies]] by plasma cells, which can cause [[hyperviscosity syndrome]], kidney damage, and [[immune suppression]].
 
== Clinical Features ==
Common symptoms include:
* Persistent [[bone pain]], often in the spine, ribs, and pelvis
* [[Fatigue]] caused by [[anemia]]
* Pathologic [[fractures]] due to weakened bone structures
* Increased susceptibility to [[infections]] due to impaired immune function
 
Patients may also present with systemic complications such as [[hypercalcemia]] and kidney dysfunction.
 
== Diagnosis ==
Diagnosis is typically made through a combination of:
* [[Blood tests]] to detect abnormal proteins (e.g., [[M protein]]) and assess kidney function
* [[Urine tests]] to identify [[Bence Jones proteins]]
* [[Bone marrow biopsy]] to evaluate plasma cell infiltration
* [[Medical imaging]] techniques like [[X-rays]], [[CT scans]], and [[MRI]] to identify osteosclerotic changes
 
In some cases, [[PET scans]] are used to assess the extent of disease spread.
 
== Associated Conditions ==
### Lymph Node Changes
In osteosclerotic plasma cell myeloma, [[lymph node]]s may exhibit changes resembling those seen in [[Castleman disease]], a rare condition characterized by abnormal growth of lymphoid tissue.
 
### POEMS Syndrome
This subtype is frequently associated with [[POEMS syndrome]], which stands for:
* **P**olyneuropathy (nerve damage causing weakness or numbness)
* **O**rganomegaly (enlargement of organs such as the [[liver]] or [[spleen]])
* **E**ndocrinopathy (hormonal imbalances, such as [[diabetes]] or [[hypothyroidism]])
* **M**onoclonal gammopathy (presence of abnormal monoclonal proteins)
* **S**kin changes (including thickening, hyperpigmentation, or increased hair growth)
 
== Treatment ==
Treatment options for osteosclerotic plasma cell myeloma include:
* **[[Chemotherapy]]**: Standard regimens to reduce plasma cell proliferation
* **[[Steroids]]**: To manage inflammation and enhance the effects of chemotherapy
* **[[Stem cell transplantation]]**: For eligible patients, this offers the potential for long-term remission
* **[[Bisphosphonates]]**: To strengthen bones and reduce the risk of fractures
* **[[Radiation therapy]]**: Targeted treatment for localized bone pain or lesions
 
== Prognosis ==
The prognosis for osteosclerotic plasma cell myeloma varies depending on the stage of the disease and response to treatment. While some patients achieve remission, others may experience disease progression. Early diagnosis and comprehensive management improve outcomes.
 
== Epidemiology ==
Osteosclerotic plasma cell myeloma is a rare condition, representing a small fraction of [[multiple myeloma]] cases. It is more commonly diagnosed in older adults and shows a slight male predominance.
 
== See Also ==
* [[Multiple myeloma]]
* [[POEMS syndrome]]
* [[Castleman disease]]
* [[Monoclonal gammopathy of undetermined significance]] (MGUS)
{{tumorstub}}
{{tumorstub}}
{{Immunoproliferative immunoglobulin disorders}}
{{Immunoproliferative immunoglobulin disorders}}
{{Chromosomal abnormalities}}
{{Chromosomal abnormalities}}
{{Authority control}}
{{Authority control}}
[[Category:Multiple myeloma| ]]
[[Category:Multiple myeloma]]
[[Category:Hematologic cancers]]
[[Category:Bone diseases]]
[[Category:Plasma cell disorders]]
[[Category:Rare diseases]]
[[Category:Oncology]]

Revision as of 02:11, 6 January 2025

Cancer of plasma cells characterized by osteosclerosis



Osteosclerotic plasma cell myeloma
Synonyms N/A
Pronounce N/A
Field Hematology and oncology
Symptoms Bone pain, fatigue, fractures
Complications Amyloidosis, kidney disease, pathologic fractures, anemia, infections, nerve damage
Onset Gradual
Duration Long term
Types N/A
Causes Unknown
Risks Obesity, genetic predisposition
Diagnosis Blood test, urine test, bone marrow biopsy, medical imaging
Differential diagnosis N/A
Prevention N/A
Treatment Steroids, chemotherapy, stem cell transplantation, bisphosphonates, radiation therapy
Medication N/A
Prognosis Variable based on stage and treatment
Frequency Rare
Deaths N/A


Osteosclerotic plasma cell myeloma is a rare subtype of plasma cell neoplasm characterized by osteosclerosis, a condition in which abnormal hardening occurs in the bones. This variant is distinct from other forms of multiple myeloma due to its association with bone formation abnormalities and fibrotic changes in the bone trabeculae.

Pathophysiology

Osteosclerotic plasma cell myeloma involves the proliferation of abnormal plasma cells within the bone marrow, which leads to the replacement of normal bone marrow elements. Unlike traditional osteolytic lesions seen in classic multiple myeloma, this subtype is marked by osteosclerosis, resulting in increased bone density. These changes are due to the interaction between cancerous plasma cells and osteoblasts, the cells responsible for bone formation.

The disease is also linked to excessive production of monoclonal antibodies by plasma cells, which can cause hyperviscosity syndrome, kidney damage, and immune suppression.

Clinical Features

Common symptoms include:

  • Persistent bone pain, often in the spine, ribs, and pelvis
  • Fatigue caused by anemia
  • Pathologic fractures due to weakened bone structures
  • Increased susceptibility to infections due to impaired immune function

Patients may also present with systemic complications such as hypercalcemia and kidney dysfunction.

Diagnosis

Diagnosis is typically made through a combination of:

In some cases, PET scans are used to assess the extent of disease spread.

Associated Conditions

      1. Lymph Node Changes

In osteosclerotic plasma cell myeloma, lymph nodes may exhibit changes resembling those seen in Castleman disease, a rare condition characterized by abnormal growth of lymphoid tissue.

      1. POEMS Syndrome

This subtype is frequently associated with POEMS syndrome, which stands for:

  • **P**olyneuropathy (nerve damage causing weakness or numbness)
  • **O**rganomegaly (enlargement of organs such as the liver or spleen)
  • **E**ndocrinopathy (hormonal imbalances, such as diabetes or hypothyroidism)
  • **M**onoclonal gammopathy (presence of abnormal monoclonal proteins)
  • **S**kin changes (including thickening, hyperpigmentation, or increased hair growth)

Treatment

Treatment options for osteosclerotic plasma cell myeloma include:

  • **Chemotherapy**: Standard regimens to reduce plasma cell proliferation
  • **Steroids**: To manage inflammation and enhance the effects of chemotherapy
  • **Stem cell transplantation**: For eligible patients, this offers the potential for long-term remission
  • **Bisphosphonates**: To strengthen bones and reduce the risk of fractures
  • **Radiation therapy**: Targeted treatment for localized bone pain or lesions

Prognosis

The prognosis for osteosclerotic plasma cell myeloma varies depending on the stage of the disease and response to treatment. While some patients achieve remission, others may experience disease progression. Early diagnosis and comprehensive management improve outcomes.

Epidemiology

Osteosclerotic plasma cell myeloma is a rare condition, representing a small fraction of multiple myeloma cases. It is more commonly diagnosed in older adults and shows a slight male predominance.

See Also

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