Paraproteinemias



Entities That Can Feature A Monoclonal Protein/M Component:

• MM

• WM

• MGUS

• MGCS

• MGRS

• Splenic Marginal Zone Lymphoma (HCV)

• CLL & MBL

• Heavy Chain disease (Gamma, Alpha & Mu)



Skin Manifestations:

• Scleromyxedema

• NXG

• Neutrophilic dermatosis

• Pyoderma gangrenosum

• Cutaneous macroglobulinosis

• POEMS: hypertrichosis, hyperpigmentation, hemangiomata, clubbing Clarkson/Systemic capillary leak syndrome

• Erythema elevatum et diutinum



Neurologic Manifestations:

• Hyperviscosity syndrome

• CVST

• Bing-Neel syndrome

• DADS-M

• POEMS (Sclerotic myeloma, lambda predominance)

• CANOMAD (Ataxia+neuropathy)

• Systemic late-onset nemaline myopathy

• Anti MAG neuropathy

• Scleromyxedema (Dermato-neuro syndrome)



MGRS:

• Fibrillar deposits:

• Amyloidosis and monoclonal fibrillary GN

• Microtubular: Immunotactoid GN & Cryoglobulinemic GN

• Crystalline: light chain proximal tubulopathy/Fanconi, crystal-storing histiocytosis, and cryocrystalglobulin GN -C3 GN

• Cast nephropathy

• TMA

• Marginal zone lymphoma



Multi-System Involvement:

• POEMS (Sclerotic myeloma↑VEGF+M component+neuropathy+Castleman+Organomegaly+↑RBC/plt+, ↑thrombosis+Pulm HTN+skin)

• Scleromyxedema (CNS+GI+CVS+joints)

• Amyloidosis (CVS+PNS+GI+Endocrine)

• TEMPI

• TAFRO



Paraproteinemia & Autoimmunity:

• -MM/WM: Vasculitis (Cryoglobulinemic, Hypo/normo-complementemic urticarial, Schnitzler syndrome, Hypergammaglobulinemic macular Vasculitis)

Autoantibodies:

• WAIHA

• CAD

• PCH

• Evans

• ITP

• Cl-NH: angioedema

• vWD

• acquired F-X def (Coagulopathy)

SMZL:

• AIHA

• Coombs (-) hemolytic anemia due to cold IgG

• Lupus anticoagulant

• IgM anti-cardiolipin

• ITP



Miscellaneous:

• Sneddon-Wilkinson disease (Subcorneal pustular dermatosis)

• Gleich syndrome (Episodic angioedema+eosinophilia)

• Pseudohyponatremia

• ↓anion gap

• CRAB

• Hyperviscosity (Thrombosis in vascular beds-renal, retinal, DVT, CVST)



By Dr. Kirtan Patolia @KirtanPatolia and Dr. Ravi Singh @rav7ks



#Paraproteinemias #Hematology #Monoclonal #Differential #Diagnosis #Oncology