Not many entities in Medicine are as intricate as Paraproteinemias

As we celebrate National Internal medicine day today, here is a 🧵exploring its various associations and complications

#MedTwitter #hematology @AnnKumfer @RebeccaEBerger @Sharminzi @rabihmgeha @DxRxEdu @rav7ks
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)

There are various others indirectly associated with the M component. More to follow
Skin manifestations

-Scleromyxedema
-NXG
-Neutrophilic dermatosis
-Pyoderma gangrenosum
-Cutaneous macroglobulinosis
-POEMS: hypertrichosis, hyperpigmentation, hemangiomata, clubbing
-Clarkson/Systemic capillary leak syndrome
-Erythema elevatum et diutinium

#dermtwitter
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)

#NeuroTwitter
Paraprotenemia & Autoimmunity #1

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

Autoantibodies:
-WAIHA, CAD, PCH, Evans, ITP
-C1-NH: angioedema
-vWD & acquired F-X def (Coagulopathy)
Paraproteinemia & Autoimmunity #2

-SMZL: AIHA, Coombs (-) hemolytic anemia due to cold IgG, Lupus anticoagulant, IgM anti-cardiolipin, ITP. Sometimes associated with HCV

-Gamma heavy chain disease/Franklin disease- RA, AIHA, ITP, SLE, Sjogren's, Myasthenia gravis, Vasculitis
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

#NephTwitter
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
Miscellaneous

-Sneddon-Wilkinson disease (Subcorneal pustular dermatosis)
-Gleich syndrome (Episodic angiodema+eosinophilia)
-Pseudohyponatremia
-⬇️anion gap
-CRAB
-Hyperviscosity (Thrombosis in vascular beds-renal, retinal, DVT, CVST)

@CPSolvers @RosenelliEM @jackpenner

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More from @KirtanPatolia

25 Oct
Guillain barre / GBS has an interesting differential diagnosis that has crucial management implications

Here is a thread based on what I have learned from my dear residents, attendings & journals

#MedTwitter #NeuroTwitter @rabihmgeha @DxRxEdu @AaronLBerkowitz @rav7ks
#1 GBS

- Infxs (C. jejuni, Mycoplasma, EBV, CMV, Zika,Influenza, HIV)
- Ascending weakness
- Cranial nerve involvement (CN 7)
- Usually no bowel/bladder disturbances
- Cyto-albuminologic dissociation and areflexia (may not manifest very early on)
- Rx - IVIG & Plasmapheresis
#2 GBS Variants and antibodies:

- AIDP
- Facial diplegia and paresthesia
- AMAN & AMSAN (GM1, GD1a)
- Phayngeal-Cervical-Brachial weakness (GT1a/GQ1b)
- Miller Fisher (GQ1b) (Areflexia + Ataxia + Opthalmoplegia)
- Bickerstaff encepahlitis (GQ1b)
- Pure Autonomic variant
Read 11 tweets
19 Oct
Adult-onset Still's disease/sJIA is beyond fascinating. The myriad of symptoms it can present with overlaps with many other disorders

Here is a thread exploring its spectrum

Any suggestions appreciated

#MedTwitter @CPSolvers @rabihmgeha @DxRxEdu @sargsyanz @rav7ks
Still's disease/sJIA classic symptoms
- Fever (Quotidian)
- Sore throat
- Synovitis/Arthralgia
- Hepatosplenomegaly
- Rash (Evanescent Salmon colored)
- Lymphadenopathy
- Anemia + Leukocytosis + Thrombocytosis + Hyperferritenemia + elevated ESR
- Rx (IL-1 inhibition)
Still's mimic #1 - Acute Rheumatic Fever

- Fever
- Sore throat (follows GAS pharyngitis)
- Arthralgia/Arthritis
- Rash (Erythema Marginatum)
- Elevated ESR
- Chorea
- Myopericarditis
Read 10 tweets

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