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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
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Pathophysiologic classification of the main monoclonal gammopathy of clinical significance-related disorders

Pathophysiologic classification of the main monoclonal gammopathy of clinical significance-related disorders
  Monoclonal immunoglobulin deposits Main characteristics of monoclonal gammopathy Main involved organ(s)
Immunoglobulin deposition in tissue*
AL amyloidosis Fibrillar
  • Lambda LC (75%), kappa LC (25%), IgM (<10%)
  • Systemic
  • Heart (80%), kidney (70%)
Type I cryoglobulinemia Microtubular/crystalline
  • IgG or IgM
  • Systemic
  • Skin +++, kidney, peripheral nerve, systemic symptoms (crystal-cryoglobulin)
Immunotactoid glomerulopathy/GOMMID Microtubular
  • CLL-like clonal B cell proliferation (50%)
  • Kidney
Acquired Fanconi syndrome Crystalline
  • Kappa LC (>90%, mostly Vk1)
  • Kidney (proximal tubulopathy)
Crystal storing histiocytosis Crystalline
  • Kappa LC
  • Systemic (kidney, cornea, joints, lymphoid tissue)
Crystalline keratopathy Crystalline
  • IgG
  • Cornea
Monoclonal immunoglobulin deposition disease (MIDD) Non-organized
  • LCDD: LC only
    • Usually kappa LC (80%, Vk1 and Vk4)
  • HCDD: truncated HC only
    • Mostly gamma (gamma-1 and gamma-3), alpha, delta
  • LHCDD: LC plus truncated HC
  • Systemic
  • Kidney (approximately 100%, glomerular and tubular basement membrane), liver (30%), heart (30%)
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) Non-organized
  • Usually IgG3
  • Kidney
Macroglobulinosis Non-organized
  • IgM
  • Skin (dermis)
Autoantibody activity
Type II mixed cryoglobulinemia Rheumatoid
  • IgM
  • Immune complex-mediated vasculitis
  • Skin +++, kidney, peripheral nerve
  • May be systemic
C1 inhibitor deficiency C1 inhibitor  
  • Angioedema
Von Willebrand (vW) disease vW factor  
  • Bleeding
Bullous skin diseases Dermo-epidermal junction (collagen VII)  
  • Skin
Xanthomatosis Various lipoproteins
  • Usually IgG
  • Cholesterol accumulation in macrophages
  • Skin and tendons
  • Other localizations (necrobiotic xanthogranumathosis)
Cold agglutinin disease Red blood cell (Ii)
  • IgM
  • Cold-induced skin manifestations plus intravascular hemolysis
IgM associated peripheral neuropathy

Myelin associated glycoprotein (MAG) +++

Gangliosides
  • IgM
  • Peripheral nerve
  • Ataxic polyneuropathy (anti-MAG)
  • CANOMAD
CAP activation
C3 glomerulonephritis

Mechanism to be determined

Autoantibody activity against CAP regulator protein (factor H) in some cases
  • IgG
  • Kidney only
Atypical hemolytic-uremic syndrome (HUS)

Mechanism to be determined

Autoantibody activity against CAP regulator protein (factor H) in some cases
  • IgG
  • Systemic
Cytokine-mediated
POEMS syndrome Vascular endothelial growth factor
  • Lambda LC (approximately 100%)
  • IgA (50%)
  • V-gamma-1 (100%)
  • Osteosclerotic bone lesions
  • Peripheral nerve (100%)
  • Various other manifestations
Unknown mechanism
Systemic capillary leak syndrome  
  • IgG, IgA (rare)
  • Systemic
TEMPI syndrome  
  • IgG
  • Systemic
Neutrophilic dermatosisΔ  
  • IgA (>80%; except Sweet's syndrome)
  • Skin +++
  • Different types and different associated manifestations
Acquired cutis laxa  
  • Usually IgG
  • Association with gamma HCDD
  • Skin +++
  • Other manifestations (lung, digestive tract)
Scleromyxedema  
  • IgG with slow electrophoretic mobility
  • Skin +++
  • Other localizations
Scleredema  
  • IgG
  • Skin only
Schnitzler's syndrome Acquired auto-inflammatory syndrome by IL-1 deregulation?
  • IgM
  • Skin +++
  • Systemic symptoms
  • Osteosclerotic bone lesions
Sporadic late onset nemaline myopathy (SLONM)    
  • Exclusively muscles (skeletal and possibly cardiac)
MGCS: monoclonal gammopathy of clinical significance; AL amyloidosis: immunoglobulin light chain amyloidosis; LC: light chain; IgM: immunoglobulin M; IgG: immunoglobulin G; GOMMID: glomerulonephritis with organized microtubular monoclonal immunoglobulin deposits; CLL: chronic lymphocytic leukemia; LCDD: light chain deposition disease; HCDD: heavy chain deposition disease; HC: heavy chain; LHCDD: light and heavy chain deposition disease; CANOMAD: Chronic Ataxic Neuropathy, Ophthalmoplegia, Monoclonal IgM protein, cold Agglutinins, and Disialosyl antibodies; CAP: complement alternative pathway; POEMS: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes; IgA: immunoglobulin A; TEMPI: Telangiectasias, Erythrocytosis with elevated erythropoietin level, Monoclonal gammopathy, Perinephric fluid collection, and Intrapulmonary shunting; IL-1: interleukin 1.
* AL amyloidosis is the most frequent type with an incidence of about 10/million inhabitants/per year in Western countries. MIDD incidence is estimated to be 10-fold lower. Other types of MGCS due to deposition of monoclonal Ig are rare (type I cryoglobulinemia, PGNMID), even scarcer (Fanconi syndrome) or exceptional (macroglobulinosis, crystalline keratopathy).
¶ A type II mixed cryoglobulinemia was observed in about 10% of patients with chronic virus C infection.
Δ Including pyoderma gangrenosum, Sweet's syndrome, subcorneal pustular dermatosis, and erythema elevatum diutinum.
Republished with permission of the American Society of Hematology, from: Fermand JP, Bridoux F, Dispenzieri A, et al. Monoclonal gammopathy of clinical significance: A novel concept with therapeutic implications. Blood 2018; permission conveyed through Copyright Clearance Center, Inc. Copyright © 2018.
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