Waldenstrom's Macroglobulinemia
- Mutation: MYD88 mutation.
- B-cell malignancy
- Cell Types affected:
- Lymphocytes, plasma cells, mast cells
- Other Name:
- Lymphoplasmacytic Lymphoma
- Definition:
- Elevated IgM (highest molecular weight).
- Serum M component
- IgM > 30 g/L
- Large IgM size → minimal renal excretion
- Only ~20% patients excrete light chains
- Clinical:
- Hyperviscosity syndrome
- d/t rouleaux formation
- Thick blood → HTN, visual issues, Nose bleed
- Peripheral neuropathy
- Due to myelin-associated glycoprotein
- Causes demyelination
- Bone marrow
- >10% infiltration with Lymphoplasmacytic cells
- Mnemonic: Wardern → Nishara
- Always in Middle of Girls (Myd 88)
- High BP (HTN)
- Masturbates (Mastocytosis)
NOTE:
Interpupillary distance → normal
ㅤ | Waardenburg Syndrome |
ㅤ | • White forehead • Piebaldism + • Dystopia canthorum • Cochlear deafness. • Heterochromia iridis. Bald (Piebald) ayittulla Wardernu (Wardenburg) Vote kodutha Aarada (Vogt Harada) |
ㅤ | Vogt Koyanagi Harada (VKH) Syndrome: |
CF | • Granulomatous Panuveitis |
Age | • Third or fourth decade |
Signs | • Sunset glow fundus • Perilimbal Vitilligo: Suiguira sign |
3 Phases | 1. Meningoencephalitic phase (Distinguished) 2. Uveitis and Choroiditis (Distinguished) 3. Leukoderma, poliosis, and alopecia |
ㅤ | Sympathetic ophthalmitis |
CF | • Granulomatous Panuveitis |
Signs | • Retrolental Flare • Dalen Fuchs nodules |
Pathology | ㅤ |
↳ Exciting Eye | • Eye that sustains initial injury. • Penetrating Trauma • Affecting ciliary body |
↳ Sympathizing Eye | • The fellow eye, not initially injured. • Develops after 2 weeks (>2 weeks) from initial trauma. |
Treatment | • Steroids |
Prevention | • Enucleation of the traumatic eye within 14 days |
- Granulomatous Panuveitis seen in
- Sympathetic Ophthalmitis
- VKH syndrome
- Wardenberg syndrome
- Wartenburg sign
- Involuntary abduction of little finger at rest
- Loss of hypothenar function → Digiti minimi
- Wartenburg syndrome
- Radial cutaneous nerve
- Also called Cheiralgia paresthetica.
- Both Warts in Hand
Type | Cause |
Posterior | • Lowe syndrome ↳ Oculo Cerebro Renal syndrome • Opacity at posterior capsule center |
Anterior | SAW Spina bifida Alport syndrome Waardenburg syndrome |
• R eye normal
• L hypochromic eye affected
Fuchs Terms | Notes |
Fuchs heterochromia iridocyclitis | Chronic AU ⇒ • U/L Diffuse Iris atrophy + Heterochromia + Posterior SCC • Painless, No redness, No posterior synechiae Positive Amsler's Sign: ◦ Bleeding into Anterior chamber on paracentesis ◦ Without trauma to Iris/Angle ◦ D/t abnormal fragile Iris Stellate Keratin Precipitates ↳ Herpetic uveitis ↳ Toxoplasmosis ↳ Fuchs Heterochromia Iridocyclitis • Young stella → Fucked () by Toxic () Herpes () Guy |
Dalens Fuchs | • Seen in Sympathetic ophthalmitis ↳ (granulomatous panuveitis) • Dalen Fucked Granny () sympathetically () |
Foster Fuchs | • In Pathological Myopia • Bleeding at macula • Fucking in Foster () home ↳ Blind child (Pathological myopia) ↳ Bled (Bleeding at macula) |
Fuchs Endothelial dystrophy | • Cornea guttata: • Wart-like excrescences on posterior cornea • Fuck her Guts→ endothelial Stages • Stage 1: Central corneal guttata that spreads peripherally • Stage 2: Corneal oedema - beaten metal-like appearance • Stage 3: Bullous keratopathy • Stage 4: Subepithelial scarring and superficial vascularization |
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