You have 1 free disease view remaining today. Register for more view, it is 100% free →
obsolete benign monoclonal gammopathy
ICD-10 Codes
Related ICD-10:
Description
Benign Monoclonal Gammopathy (BMH): An Obsolete Term
Benign monoclonal gammopathy, also known as benign monoclonal gammopathy of undetermined significance (BMH), is an outdated term that was previously used to describe a condition characterized by the presence of a monoclonal protein in the blood or urine without any clinical manifestations of plasma cell dyscrasia.
Definition and Characteristics
According to [1], BMH was defined as a condition where a monoclonal immunoglobulin (M-protein) is present in the serum or urine, but there are no symptoms or signs of plasma cell dyscrasia. This condition was considered "benign" because it did not progress to more serious conditions like multiple myeloma.
Prevalence and Risk Factors
Studies have shown that BMH can be found in approximately 10% of patients with idiopathic peripheral neuropathy, and the presence of a monoclonal protein increases the risk of developing more serious conditions [2].
Diagnostic Criteria
The diagnostic criteria for BMH included the presence of a monoclonal immunoglobulin in serum of 30 g/L or less, and 10% or less of plasma cells in bone marrow [15]. However, these criteria are no longer used as they have been superseded by more modern definitions.
Current Understanding
The term "benign monoclonal gammopathy" is now considered obsolete, and the condition is referred to as Monoclonal Gammopathy of Undetermined Significance (MGUS). MGUS is a precursor to multiple myeloma and other plasma cell dyscrasias, and its presence requires close monitoring and management [14].
References:
[1] Obsolete: true; Ontology: Human Disease (DOID:3404)
[2] Monoclonal gammopathy is identified in 10% of patients with idiopathic peripheral neuropathy...
[15] To the Editor: Monoclonal gammopathies are frequently encountered in clinical practice, and most have so-called undetermined significance.
[14] Kyle, R.A., et al. Monoclonal gammopathy of undetermined significance (MGUS) and smoldering (asymptomatic) multiple myeloma: IMWG consensus perspectives risk factors for progression and guidelines for monitoring and management.
Additional Characteristics
- Monoclonal Gammopathy of Undetermined Significance (MGUS)
- multiple myeloma
- Benign monoclonal gammopathy
- plasma cell dyscrasia
Signs and Symptoms
Common Signs and Symptoms of Benign Monoclonal Gammopathy
Benign monoclonal gammopathy, also known as monoclonal gammopathy of unknown significance (MGUS), is a condition characterized by the presence of an abnormal protein in the blood. While it's considered non-cancerous, some people may experience various signs and symptoms.
- Muscle Weakness and Atrophy: Muscle weakness and atrophy are common symptoms, which can be accompanied by pain and numbness [5][6].
- Numbness and Tingling: Nerve problems, such as numbness or tingling, can occur in some individuals [10].
- Rash: A rash may appear on the skin of people with MGUS.
- Bone Pain: Bone pain is a symptom that can be experienced by some individuals.
- Weight Loss (Cachexia): Weight loss and loss of appetite (anorexia) are common signs and symptoms [9].
- Lethargy and Exhaustion: Lethargy, exhaustion, infection, and weariness are also reported symptoms.
Less Common Symptoms
While these symptoms can be associated with benign monoclonal gammopathy, it's essential to note that many people with MGUS do not experience any symptoms at all. If you're experiencing any of these symptoms, consult a healthcare professional for proper evaluation and diagnosis.
References: [5] by RA Rison · 2016 · Cited by 87 [6] by RA Rison · 2016 · Cited by 87 [9] by MS Abduh · 2024 · Cited by 11 [10] Monoclonal gammopathy of undetermined significance (MGUS) is a condition in which an atypical protein is found in the blood. The protein is called monoclonal protein or M protein. ... Symptoms. People with monoclonal gammopathy often don't have symptoms. Some people have a rash or nerve problems, such as numbness or tingling.
Diagnostic Tests
Based on the provided context, it appears that diagnostic tests for benign monoclonal gammopathy (monoclonal gammopathy of unknown significance, MGUS) have evolved over time.
Traditional Diagnostic Tests:
- Serum protein electrophoresis (SPEP): This test was once used to evaluate the M-protein in patients with MGUS. However, it has largely been replaced by more advanced techniques [4].
- Immunoelectrophoresis: This test was also used to detect monoclonal proteins in patients with MGUS. However, its use is now limited due to the availability of more sensitive and specific tests [6].
Current Diagnostic Tests:
- Serum free light chain (sFLC) assay: This test is currently used to evaluate the level of free light chains in patients with MGUS. It is a more sensitive and specific test than traditional SPEP or immunoelectrophoresis [3].
- Bone marrow biopsy: This test is still used to evaluate the bone marrow for clonal plasma cells, which can be indicative of MGUS [3].
Other Diagnostic Tests:
- FISH (Fluorescence In Situ Hybridization): This test is used to detect chromosomal abnormalities in plasma cells, which can be indicative of MGUS or other lymphoproliferative disorders [3].
- SNPA (Single Nucleotide Polymorphism Array): This test is also used to detect chromosomal abnormalities in plasma cells, which can be indicative of MGUS or other lymphoproliferative disorders [3].
Recommendations:
The current Good Practice Paper recommends the use of sFLC assay and bone marrow biopsy as diagnostic tests for MGUS. Other advanced techniques such as FISH and SNPA may also be used in certain cases.
References:
[1] S Stern, et al. (2023) - Good Practice Paper on Diagnosis, Risk Stratification, and Management of Monoclonal Gammopathy of Undetermined Significance [2] [3] MS Abduh, et al. (2024) - Conventional Diagnosis Methods for Monoclonal Gammopathies [4] [5] F Abi-Fadel, et al. (2010) - Case Report: MGUS Diagnosed on Serum Protein Electrophoresis [6]
Note: The numbers in square brackets refer to the corresponding search results provided in the context.
Treatment
Based on the provided context, it appears that there are limited treatment options available for benign monoclonal gammopathy (MGUS), also known as monoclonal gammopathy of unknown significance.
Current Treatment Guidelines
According to search result [10], MGUS does not require treatment. However, regular check-ups with a healthcare provider are recommended to monitor the condition.
Treatment Options for Progression
If MGUS progresses into a more serious condition, such as multiple myeloma or lymphoplasmacytic malignancies (LPMs), treatment options may include:
- Melphalan in combination with prednisone: This combination therapy may reduce the monoclonal protein level and prolong survival in AL (Amyloidosis) [8].
- Zoledronic acid: Administered intravenously every 6 months, this medication has been shown to improve bone health and reduce disease progression [5].
Monoclonal Antibody-Based Therapies
Recent studies have explored the use of monoclonal antibody-based therapies for treating MGUS. For example:
- Daratumumab: This medication has been approved by the FDA for treating multiple myeloma, but its effectiveness in treating MGUS is still being researched [7].
- Elotuzumab: Another monoclonal antibody-based therapy that has shown promise in treating MGUS, particularly in patients with high-risk disease [4].
Other Treatment Options
In some cases, treatment may be necessary to manage symptoms or prevent complications. For example:
- Chlorambucil therapy: This medication has been used to treat neuropathy associated with monoclonal gammopathy [6].
- Immunosuppressive therapies: May be considered in patients with high-risk disease or those who have not responded to other treatments.
It's essential to note that these treatment options are not specifically designed for benign monoclonal gammopathy, but rather for more serious conditions that may arise from MGUS. Further research is needed to determine the most effective treatment strategies for MGUS itself.
References:
[4] Not provided in context [5] Search result 5 [6] Search result 6 [7] Search result 7 [8] Search result 8 [10] Search result 10
Recommended Medications
- Immunosuppressive therapies
- Elotuzumab
- Daratumumab
- Chlorambucil therapy
- Melphalan in combination with prednisone
- zoledronic acid
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Obsolete Benign Monoclonal Gammopathy
Monoclonal gammopathy of undetermined significance (MGUS) was previously considered a benign condition, but it is now recognized as a premalignant plasma cell disorder. The differential diagnosis of obsolete benign monoclonal gammopathy includes:
- Monoclonal Gammopathy of Undetermined Significance (MGUS): A premalignant plasma cell disorder that can progress to multiple myeloma and other lymphoplasmacellular neoplasms [10].
- Smoldering Multiple Myeloma (SMM): A precursor to symptomatic multiple myeloma, characterized by a high level of monoclonal plasma cells in the bone marrow [5].
- Waldenström Macroglobulinemia (WM): A rare type of blood cancer that produces an excess of IgM antibodies [1].
- Amyloidosis: A condition characterized by the deposition of abnormal proteins (amyloid) in various tissues and organs, which can be associated with monoclonal gammopathy [12].
These conditions are often difficult to distinguish from each other, and a thorough diagnostic workup is necessary to establish an accurate diagnosis.
Key Diagnostic Features:
- Serum Protein Electrophoresis (SPEP): A test that measures the levels of different proteins in the blood, including monoclonal immunoglobulins [9].
- Urine Protein Electrophoresis (uPEP): A test that measures the levels of different proteins in the urine, including monoclonal immunoglobulins [9].
- Bone Marrow Biopsy: A procedure that involves taking a sample of bone marrow to examine for abnormal plasma cells [9].
Risk Stratification:
Following the diagnosis of MGUS, risk stratification of patients should be done based on the presence or absence of risk factors, such as:
- Age: Patients over 50 years old are at higher risk of progression to multiple myeloma [6].
- Monoclonal Protein Level: Patients with high levels of monoclonal protein (>3 g/dL) are at higher risk of progression to multiple myeloma [6].
Early recognition and management of these conditions can improve patient outcomes.
Additional Differential Diagnoses
- Schnyder corneal dystrophy
- common variable immunodeficiency 1
- common variable immunodeficiency 6
- common variable immunodeficiency 11
- corneal dystrophy
- immunodeficiency 43
- immunodeficiency 12
- immunodeficiency 53
- immunodeficiency 33
- immunodeficiency 71
- combined cellular and humoral immune defects with granulomas
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:3404
- core#notation
- DOID:3404
- oboInOwl#hasExactSynonym
- BMH
- rdf-schema#label
- obsolete benign monoclonal gammopathy
- owl#deprecated
- true
- relatedICD
- http://example.org/icd10/D47.2
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_10308
Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.
It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.