Thread by @WuidQ

1/3
Sorry guys for the short hiatus. But I’m back! Let’s start with an #idgrandrounds case (as always, patient info modified for privacy)

👉 36/M w/ HIV, p/w GTC seizure. No prodrome. No fever, chills, headache, vision changes

@LeMiguelChavez @NNolanMD @Jncherabie @arauseomd

2/3
2 mos ago, admitted +confusion. That time, CD4 40, VL 4M. Extensive w/u (-): n/l brain MRI, LP (0 WBC, n/l TP/gluc), (-)ID w/u including crypto, JC, EBV, CMV, Toxo. Toxo IgG(-). CT: n/l. ART started

Current admission:
Exam: T 37. Somnolent, no deficits.
Labs: CD4 380, VL 7K

3/3
Brain MRI: 4 ring-enhancing masses surrounded by edema L frontal lobe. LP: 10 WBC, (-)Toxo, EBV, JC, CMV, VDRL, AFB. Fungal(-). CT: scattered lung nodules.
No prior OI, (-)IGRAs,(-)drug use, (-)exposure/travel

Thoughts? @TxID_Edu @VarunPhadke2 @TMcCarty2010 @BradCutrellMD

1/13
Case resolution:
▪️BAL(-); nodules too small for bx
▪️Brain bx: angioinvasive, polymorphic lymphocytic infiltrates (atypical B, T cells) w/ areas of necrosis, EBV+

Diagnosis: Lymphomatoid granulomatosis (LG), IRIS

ART continued + rituximab 👉 significant improvement

2/13
Let’s review the schema we talked about previously 👉 syndromic approach to HIV OI that begins w/ knowing the CD4 count and focusing on the predominant signs & symptoms👇

3/13
The timeline of our patient’s illness can be represented as:

Point A (CD4 40, confusion, neg w/u) ➡️ ART ➡️ Point B (increased CD4, seizure, rim enhancing brain lesions)

More clues are available in Point B so let’s zoom there.

4/13
From the schema, we think of common processes (e.g. bacterial abscess), primary CNS lymphoma (PCNSL) & Toxo. IRIS has been reported in both PCNSL & Toxo.

✔️Multiple rim-enhancing lesions favor Toxo (PNCSL 70% solitary)

But what do we do w/ the (-) Toxo testing?

5/13
A (-)serology makes Toxo unlikely but not impossible. A (-)PCR also makes it unlikely but bear in mind that PCR testing is only 50% Sn. bit.ly/3eaxLZT

One could argue that our patient could be empirically Tx for Toxo & observed for clinical & radiologic response.

6/13
What do we know of LG? Let’s build the illness script.

❗️Epi:
▪️Occurs in immunocompromised persons (HIV, transplant, congenital). 20+ cases in HIV bit.ly/2TuIYwE
▪️All age groups, slight male predominance

7/13
‼️Clinical:
▪️Pulm > CNS/skin
▪️Cough & fever, most common SSX
▪️Multiple lung nodules on imaging 👇 (Image 1) bit.ly/2WWcrkY
▪️Skin (40%). Nodules, papules, ulcers 👇(Image 2) bit.ly/2TxVjjs

8/13
▪️ CNS (30%). Rim-enhancing lesions, usually multiple (unlike PCNSL in which lesion has homogenous enhancement and is soliary)
▪️Other organs: kidney, liver (nodules on imaging)
▪️Lymph nodes & spleen rarely involved ⚡️⚡️⚡️

9/13
❗️Time course:
▪️ Subacute to chronic illness much like many diseases that affect immunocompromised patients
▪️Can remit-recur/persist over months before dx 👇 bit.ly/3e9AgLX (yes, it’s from #fauci ❤️)

10/13
Mechanism:
▪️Unknown but related to abnormal response to EBV
▪️Different EBV-associated malignancy thought to arise depending on the stage of B cell dev’t arrested 👇bit.ly/2WV799y

11/13
‼️In summary, LG is an ID mimic ⚡️resembles lymphoma but differs from it (13% develop lymphoma):
▪️Polymorphic B & T cell infiltrate
▪️Predilection for vascular invasion
▪️Necrosis (“granulomatous”)
▪️Lack of spleen or LN involvement
▪️vs PCNSL (multiple, rim-enhancing)

12/13
Add LG in your DDX for immunocompromised patients w/:
▪️Fever + lung nodules (together w/ fungal, AFB, etc)
▪️Lung + brain (+/- skin). We’ve talked about this before 👇

13/13
Add LG in your DDX of brain mass lesions in HIV (+Chagas👇)

LG can resolve w/ ART alone bit.ly/2LU0QwJ or +rituximab.

LG can be a/w IRIS: bit.ly/36qiC3T bit.ly/3gchmWF. In our patient, lesions could be + in Point A & enhanced in Point B w/ IRIS