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
