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Nov 7, 2022 โ€ข 12 tweets โ€ข 6 min read โ€ข Read on X
Impressing ๐˜พ๐™ค๐™ฃ๐™จ๐™ช๐™ก๐™ฉ๐™–๐™ฃ๐™ฉ ๐™ค๐™ฃ ๐™๐™ค๐™ช๐™ฃ๐™™ โ“

Residents use the fancy diagnosis and terms like
๐‡๐‹๐‡ - Hemophagocytic lymphohistiocytosis

A ๐Ÿงต on what is ๐‡๐‹๐‡ and how to diagnose it
#MedTwitter
#HLH
#MedEd
#usmle Hemophagocytosis
๐Ÿ“Hyperinflammatory syndrome caused by โฌ†๏ธcytokine release

๐Ÿ“ Either due to genetic or acquired overactivation of CD8 T cells and natural killer (NK) cells

โœด๏ธ Hemophagocytosis is neither sensitive nor specific for HLH

๐Ÿ…ฒ๐Ÿ…ป๐Ÿ…ฐ๐Ÿ†‚๐Ÿ†‚๐Ÿ…ธ๐Ÿ…ต๐Ÿ…ธ๐Ÿ…ฒ๐Ÿ…ฐ๐Ÿ†ƒ๐Ÿ…ธ๐Ÿ…พ๐Ÿ…ฝ ๐Ÿ‘‡
Lets discuss one of the ๐–ˆ๐–†๐–˜๐–Š ๐–˜๐–ˆ๐–Š๐–“๐–†๐–—๐–Ž๐–”๐–˜ that came to AIIMS:

24,๐Ÿ’ƒ, without any Co-morbidties

C/O Fever, Malaise, Sore throat, Headache

๐Ž๐ง ๐„๐ฑ๐š๐ฆ๐ข๐ง๐š๐ญ๐ข๐จ๐ง :
๐Ÿ”ธHR ~125/min ๐Ÿ”บ
๐Ÿ”ธSevere Pallor
๐Ÿ”ธCervical Lymphadenopathy
๐Ÿ”ธPetechial Rash

Investigation๐Ÿ‘‡
Peripheral Smear ๐Ÿ‘‡ - Atypical lymphocytes {Downey Cells}
๐Ÿ“Palpable enlarged spleen
๐Ÿ“ Ctyopenias Worsening
๐Ÿ“ TG - 402 ๐Ÿ“ˆ
๐Ÿ“ Ferritin - 3K๐Ÿš€

After suspicion of ๐—˜๐—•๐—ฉ, PCR was sent which came out to be โž•ve โ™€๏ธ

A Diagnosis of 2' ๐‡๐‹๐‡ was made
๐ƒ๐ข๐š๐ ๐ง๐จ๐ฌ๐ข๐ฌ : Challenging
๐Ÿ”ธNonspecific symptoms
๐Ÿ”ธOverlapping features with sepsis and hematologic malignancy
๐Ÿ”ธSame disorders can trigger HLH

๐Ÿ…ฑ๐Ÿ†„๐Ÿ†ƒ

Essential as without Rx, ๐‡๐‹๐‡ is FATAL๐Ÿ”ด

๐˜ฟ๐™ž๐™–๐™œ๐™ฃ๐™ค๐™จ๐™ฉ๐™ž๐™˜ ๐˜พ๐™ง๐™ž๐™ฉ๐™š๐™ง๐™ž๐™– ๐Ÿ‘‡
๐Ÿ…ท - ๐Ÿ†‚๐Ÿ…ฒ๐Ÿ…พ๐Ÿ†๐Ÿ…ด
๐Ÿ“Retrospective data of 312 adult population with non-familial HLH was used to design the score

๐Ÿ“The best cutoff value: 169 (Sn-93%, Sp-86%)

๐Ÿ”ธ"HLH-2004 criteria" > H-Score - ๐˜€๐—ถ๐—บ๐—ถ๐—น๐—ฎ๐—ฟ ๐˜€๐—ฒ๐—ป๐˜€๐—ถ๐˜๐—ถ๐˜ƒ๐—ถ๐˜๐˜† ๐—ฏ๐˜‚๐˜ ๐—ต๐—ถ๐—ด๐—ต๐—ฒ๐—ฟ ๐˜€๐—ฝ๐—ฒ๐—ฐ๐—ถ๐—ณ๐—ถ๐—ฐ๐—ถ๐˜๐˜†
Investigations to be sent ๐Ÿ‘‡
๐Ÿฆ  Associated with HLH

๐•๐ข๐ซ๐š๐ฅ > ๐๐š๐œ๐ญ๐ž๐ซ๐ข๐š๐ฅ > ๐…๐ฎ๐ง๐ ๐š๐ฅ

doi.org/10.1016/S0140-โ€ฆ
๐Œ๐š๐ง๐š๐ ๐ž๐ฆ๐ž๐ง๐ญ of sHLH ๐Ÿ‘‡

doi.org/10.1016/S2665-โ€ฆ
Familial HLH in Adults ๐Ÿ‘‡
doi.org/10.1182/bloodaโ€ฆ

Two most common genes seen in "adult" HLH are ๐—ฆ๐—ง๐—ซ๐—•๐—ฃ๐Ÿฎ and ๐—ฅ๐—”๐—•๐Ÿฎ๐Ÿณ๐—”
๐ˆ๐ง๐๐ข๐œ๐š๐ญ๐ข๐จ๐ง๐ฌ ๐จ๐Ÿ ๐†๐ž๐ง๐ž๐ญ๐ข๐œ ๐“๐ž๐ฌ๐ญ๐ข๐ง๐ 
๐Ÿ”ธEarlier onset
๐Ÿ”ธRecurrent HLH
๐Ÿ”ธRefractory HLH
๐Ÿ”ธFamily History
๐Ÿ”ธConsanguinity
๐Ÿ”ธAlbinism
๐Ÿ”ธWithout an apparent trigger or predisposing disease
Hope you find it useful๐Ÿ™

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More from @CardioDocDexter

Nov 15, 2022
๐Ÿ†˜59 year male smoker
k/c/o COPD, DM, HTN with DCMP (EF=20%) palpitations and dizziness x 2 days

O/E : B/L crepts +, rest

ECG๐Ÿ‘‡

#Medtwitter #MedEd2022 #ECG #CardioEd Image
What is the likely rhythm ?
Answer - ๐˜ผ๐™ฉ๐™ง๐™ž๐™–๐™ก ๐™๐™ž๐™—๐™ง๐™ž๐™ก๐™ก๐™–๐™ฉ๐™ž๐™ค๐™ฃ

๐Ÿ“Absent discrete P-waves
๐Ÿ“Fibrillatory waves (f-waves)
๐Ÿ“Irregularly irregular QRS complexes Image
Read 6 tweets

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