Our ecosystem also serves as a Jarvis training model and all characters have been deidentified and anonymized to protect their privacy and remove PHI.
Major medical cognition EQ churning points in the transcripts probably are the introduction to this 14 year old bright school boy in rural India with his final PaJR entry by PaJR Jarvis 2020 informing us of his death as received through his father's phone call and the flashbacks containing the good times Jarvis shared with the boy (who got him a special food treat for taking care of him described somewhere in the middle of the transcript) and rest are medical cognition IQ churning points.
Much of this boy's actual EQ transcripts are left unsaid such as the moments he enjoyed with his family (especially after the weight of his spleen was removed), inspite of all the suffering he endured through his illness journey shunted throughout various hospital beds in rural and urban Hyderabad.
He felt breathless suddenly it seems was taken to a local hospital and he expired it seems sir
[11/11, 7:52 AM] Rakesh Biswas: Sepsis that was difficult to withstand post splenectomy?
He even went to his grandmothers house the previous day and was alright it seems sir
[8/30/2022, 6:23 PM] PaJR Jarvis 2019:
When this boy had jaundice initially he was managed by a pediatrician ( who was his relative) ,and he advised to get them basic investigations. They repeated CBP ,LFT and found that his bilirubin was increasing and hemoglobin was decreasing,that's when he referred to us .
On initial investigation and on background history of chronic diarrhoea secondary to malabsorption, and thyroid disease .with moderate splenomegaly and coombs positive we thought some autoimmune mediated hemolysis / secondary to vit B12 deficiency .so we started him on steroid and vit B12 ,and his pancytopenia resolved and then discharged.
[8/30/2022, 6:24 PM] PaJR Jarvis 2019: His USG abdomen showed moderate splenomegaly at that time of presentation
[8/31/2022, 6:10 PM] Rakesh Biswas: A CASE OF UNCERTAINITY?
Is there any case that is certain. Better remove this from the title.
Uncertainty exists only if we think. Previously there was no uncertainty but of late we have started thinking
[8/31/2022, 6:23 PM] Metapsychist Number 1 Kims 2015: Can overdosage of thyroxine cause weight loss?
[8/31/2022, 6:34 PM] Rakesh Biswas: @PaJR Jarvis 2020 Did we sort out his thyroxine per kg Dose? Is his current Dose ok or high?
[8/31/2022, 6:46 PM] PaJR Jarvis 2020a: In congenital hypothyroidism maximum dose given can be 8mcg/kg/day sir.
[8/31/2022, 6:48 PM] Rakesh Biswas: What is our patient's weight?
[8/31/2022, 7:01 PM] PaJR Jarvis 2020a
: 45kgs sir
[8/31/2022, 7:02 PM] Rakesh Biswas: How much is the Dose coming to as per the data you shared (not the congenital hypothyroidism where you didn't share any evidence yet)?
[8/31/2022, 7:02 PM] PaJR Jarvis 2020a
: Am searching for maximum dose in autoimmune thyroiditis sir. But i could nt find an appropriate article sir
[8/31/2022, 7:04 PM] PaJR Jarvis 2020a
: Max dose according to weight is 2.5 mcg/kg/day which is around 112.5 MCG/day sir
[8/31/2022, 7:30 PM] Rakesh Biswas: And since when is he on 200 mcg per day?
[8/31/2022, 7:44 PM] PaJR Jarvis 2020
: Since approx 3 yrs sir
[9/2/2022, 7:03 PM] PaJR Jarvis 2020a
: Apart from splenomegaly
We couldn’t find anything
Going by today’s endoscopy
[9/2/2022, 7:04 PM] Rakesh Biswas: Strange that you say this after doing his ultrasound. What were the portal and splenic vein diameters that you saw? Can they be explained by any other condition?
[9/2/2022, 7:05 PM] Rakesh Biswas: Let's not spam the patient advocates in the PaJR groups but rather spam the 2017 interns
[9/2/2022, 7:05 PM] PaJR Jarvis 2020
: That’s what sir
Dilated splenic
And portal vein
Is this a noise or signal ?? 😅
[9/2/2022, 9:58 PM] PaJR Jarvis 2020
: They want to get discharged itseems sir tomorrow
[9/2/2022, 10:02 PM] Rakesh Biswas: Share the deidentified summary in the case report link.
[9/2/2022, 10:02 PM] PaJR Jarvis 2020
: Yes sir
[11/15/2022, 11:18 AM] PaJR Jarvis 2020
: Inputs from ex elite stark jarviites of Hyderabad on this boy :
[11/14, 10:50 PM] Rakesh Biswas: Can we utilize this group's "group think" to help a 14 year old boy currently with us?
He has hypothyroidism since 2016 currently on 200 mcg of thyroxine per day along with a massive splenomegaly and dilated spleno portal vein more than 1.5 cm.
We had sent him to NIMs for portal pressure measurements and a TIPs liver biopsy if possible to rule in NCPF.
He was seen by Gastro OPD in NIMs and treated as tropical splenomegaly and adviced splenectomy for which he has returned to us. They also got the bone marrow biopsy which suggested hypersplenism. His WBC counts are 1500 and platelets 20,000
Would a splenectomy be safe without a portal spleno renal shunt? Would be looking forward to learning from the group inputs here.
More sequential information about the patient is in his working case report link here :
[11/14, 11:01 PM]: Stark Jarvis 1
Clinical picture is looking more like an NCPF ,
Upper GI endoscopy to rule out varices
LFT to have priliminary idea about portal biliopathy
Good clinical history to rule out symptomatic hypersplenism And growth retardation
CT Portovenography and look for splenic and renal vein diameters
[11/14, 11:02 PM]: SJ 2
If there is an indication
Proximal spleno renal shunt might be the choice of surgery
[11/14, 11:14 PM] : SJ 3
With advances in Interventional Radiology, may be Gel Foam occlusion of the main splenic artery can be attempted.....
[11/14, 11:14 PM] :
[11/14, 11:22 PM] : SJ 4
This case represents forward flow portal hypertension due to a large spleen and excess blood flowing through the routine splenoportal axis. In cases where liver dysfunction is not there, cirrhosis is excluded, there will be no need for a splenorenal shunt. Simple splenectomy will reduce the pressures. However if cirrhosis or NCPF is there, they would need a shunt since resistance is at sinusoidal level in the liver in both these conditions and it would persist even after splenectomy.
We would need an endoscopy to look for portal hypertension features like varices, portal hypertensive gastropathy. It's always better to get a liver biopsy and HVPG measurement before proceeding further. Since platelet count is low and cannot support a liver biopsy, we can ask Interventional radiologist to do a trans jugular liver biopsy and measure HVPG (hepatic venous portal gradient) in same sitting.
Splenic artery embolization is a good technique but with a massive spleen it might create troubles like massive splenic infarct causing pain, infection, abscess, etc. Better to undergo splenectomy than embolization in such massive spleens.
[11/14, 11:28 PM] : SJ5
Ncpf vs hypersplenism
Requires minimum of splenectomy
Preop biopsy can give an idea of ncpf
Intraop portal pressures can be measured post splenectomy
Splenic artery embolization has inferior results compared to conventional splenectomy
Does not work well with massive splenomegaly and has attendant severe complications when done in massive splenomegaly
Thanks
[11/14, 11:29 PM]: SJ 6
Shunt should be considered if post splenectomy pressures are significantly high post splenectomy
[11/15, 5:32 AM] : SJ 7
1. Crucial information needed is presence or absence of oesophago-gastric varices and their grade.
2. Symptomatic massive splenomegaly with hyperdynamic circulation, no/insignificant varices and hypersplenism - *Simple splenectomy*
3. Symptomatic massive splenomegaly with hypersplenism and varices - *Splenectomy with shunt*
4. Asymptomatic splenomegaly and asymptomatic well-tolerated hypersplenism - *No surgery*
4. No role for shunt surgery in pre-primary prophylaxis. Some role in selected patients in primary prophylaxis and definite role in secondary prophylaxis in non-cirrhotics.
5. HVPG as a marker of PH and as a predictive factor for variceal development in cirrhotics and for chemoprophylaxis is well-recognized. *Any evidence in noncirrhotics?*
[11/15, 6:43 AM] SJ 8 : This young male has
Symptomatic massive splenomegaly with hypersplenism
as SJn suggested (point no 2) - SIMPLE SPĹENECTOMY is enough.
There are no varices either on endoscopy or in imaging.
Points for discussion
1. Coomb’s positive - patient
was given steroids ? AIHA
2. Thyroid dysfunction-
should we rule out
Hashimoto’s?
3. Should we consider
Evan’s syndrome
Overall a Simple Splenectomy will be useful in most of there scenarios.
[11/15, 7:26 AM] SJ 8
: In addition to TPO antibodies and ensuring he is taking tablet on empty stomach,we need to consider "consumption component" due to organomegaly ( as he is requiring high dose of levothyroxine).
[11/15, 7:27 AM] SJ 8 : As long as he is euthyoid,fit for surgical procedure,if no other autoimmune conditions.👍🏻
[11/15, 8:06 AM] SJ 9: is it not necessary to rule out ncpf before we opt for splenectomy , how far splenectomy be beneficial in ncpf or any portal vien thrombosis
[11/15, 8:41 AM] SJ 10 : This patient is having
Symptomatic massive
splenomegaly with
hypersplenism.
No evidence of any portal vein thrombus in the imaging.
No varices on UGI scopy.
( if it is EHPVO - Splenectomy in this patient will be almost a cure).
Simple splenectomy will be a practical & beneficial to the patient.
A wedge liver biopsy during splenectomy will be useful for long term follow up.
Even if it is NCPF - when there are no signs of any varices - role of prophylactic shunt surgery is questionable.
[11/15, 8:52 AM] SJ 11 :
Young boy
With splenomegaly and hypersplenism
Definitely need evaluation
for AIHA and Evans
Such a picture can be seen in inborn errors of immunity
Ex CVID, autoimmune lyphoproliferative syndrome( Evans like picture would be seen) , which are often missed
Before exploring surgical options
Immunoglobulin level estimation, double negative T cells estimation is important .
[11/15, 8:58 AM] SJ 11: I agree with you completely about the further work up for autoimmune etc…
But at this point of time for this young boy with symptomatic Massive splenomegaly and hypersplenism - splenectomy is a good option.
About the work up either you can do pre-operative or post-operative.
Follow up accordingly.
[11/15, 9:04 AM] SJ 12: Any surgery in portal hypertension except Liver transplant is at best a palliative procedure, intended to reduce pressure in the most significant locations in the portal venous system, in order to prevent life-threatening oesophago-gastric bleeds and problems of portal biliopathy or symptoms of massive symptomatic splenomegaly and splenic sequestration.
[11/15, 9:12 AM] SJ 7: I think in a 14 year young boy
Splenectomy alone may cause oesophago-gastric varices and portal biliopathy in the future
So adding shunt procedure would be beneficial
As the natural course following splenectomy alone is unpredictable
[11/15, 9:20 AM] SJ 7: In the 80s and 90s, the staging of Hodgkins Lymphoma was through a formal staging lapatomy, that included splenectomy.
Over the past 40 years, we have followed these children.
So far we have not encountered any evidence of portal hypertension in a single patient (now adults).
Strangely, pneumococcal pneumonia too appears to be not so common in splenectomised Indians.
I will be very happy to have a peripheral smear of this patient.
It will be a good teaching slide...reflecting all the senescent and defective cells which would have otherwise been removed by the spleen.
[11/15, 9:23 AM] SJ 10: The only patient I lost to was an adult with an inborn error in erythroid membrane, splenectomised on advice at CMC.
He manifested post operatively and over the next couple of years with arterial thrombi.
And, finally succumbed to massive pulmonary embolism.
[11/15/2022, 11:56 AM] Rakesh Biswas: My response to their responses :
Thanks for these very useful inputs that will go a long way to help this 14 year old, bright school boy from Nalgonda and also demonstrate the positive power of group think in this August group.
Few of you have also gone through his details shared in the working case report link and already some of the queries raised here have been answered such as the absence of varices etc.
If we go through the working case report link we also find that he does have an immune mediated inflammation going on in multiple organ systems since childhood involving intestine, thyroid, RBCs etc and presented with features of hypersplenism and severe pancytopenia this time. We may find a lot more detail about his childhood sequence of events as well as family history in the working case report link shared above to understand his immune dysfunction better (although it's still a very sketchy description now at best).
As pointed out by many here, we too were apprehensive about our planned splenectomy causing his current portal pressures into getting redirected to his currently non existing varices or even peritoneal capillaries causing bleeds and ascites that is absent at present but as SJn clarified , this current Portal hypertension could be due to splenic flow redistribution so a simple splenectomy may suffice but yes an HVPG and possibly TIPs liver biopsy would be useful to reduce the diagnostic uncertainty before proceeding with the splenectomy and this was also the reason we had sent him to NIMs.
Our rural medical college surgeons can do the splenectomy (also perhaps with a per operative liver biopsy) at low-cost and like all our rural patients here, they too may not be able to afford much.
Following the above discussion it appears that we may not be able to reduce the diagnostic uncertainty around his portal pressures and NCPF any better than this so it may be best to quickly offer him the splenectomy (with per operative liver biopsy) here in our rural medical college?
Or should he be operated in a set up where one can check his perop portal pressures and offer a PSRS (proximal spleno renal shunt) decision right on table (although again it may become unaffordable perhaps)?
Will be glad to learn more from your inputs.
[11/16/2022, 2:32 PM] PaJR Jarvis 2020
: Sir
Should we go ahead with splenectomy and liver biopsy ??
His parents are asking for splenectomy too sir
[11/16/2022, 2:35 PM] Rakesh Biswas: Yes let's. Off course only if our surgeons agree
[11/22/2022, 11:51 AM] PaJR Jarvis 2020
Sir
BAL is done
I have found about
Beta d glucan and galactamine tests
It will costing them 10000 and 8400 separately
[11/22/2022, 12:01 PM]
PaJR Jarvis 2018
: What is the latest on him ? Why was a BAL done ?
[11/22/2022, 12:21 PM] Rakesh Biswas: Poor sensitivity 50% and specificity 80% for a test costing 10,000
[11/22/2022, 12:22 PM] Rakesh Biswas: The next question is if these biomarkers are better in distinguishing colonizing Aspergillus from pathogens?
For that we need to review other literature
[11/22/2022, 1:16 PM] PaJR Jarvis 2020
[11/22/2022, 1:18 PM] PaJR Jarvis 2020
: Good afternoon sir
Yesterday we had a CDM (clinico diagnostic meet)
His HRCT was showing GGO’s
?? Halo sign
They want to rule out fungal etiology sir
They asked us to get the BAL done before surgery
[11/22/2022, 1:49 PM] PaJR Jarvis 2018
: There is something sinister here perhaps ? Are you considering any other immunodeficiency syndromes ?
Our recently accepted publication tells that is badly underdiagnosed
[11/22/2022, 2:04 PM] PaJR Jarvis 2020a
: Immunoglobulin electrophoresis was normal i guess sir. Please validate this @PaJR Jarvis 2020
[11/22/2022, 2:16 PM] PaJR Jarvis 2018
: What about Neutrophil disorders ? Phagocytic disorders ? T cell disorders ? And combined immunodeficiencies ?
[11/25/2022, 4:41 PM] PaJR Jarvis 2020
: Good afternoon sir
[11/25/2022, 4:41 PM] : PaJR Jarvis 2020
The FNAC report of lymph node and thyroid is showing mets sir
[11/25/2022, 4:50 PM] PaJR Jarvis 2020:
Thyroid is suggestive of bethesda cat 2
[11/25/2022, 6:03 PM] Rakesh Biswas:
Is lung the primary.
Please update this in the case report and let me again share it in the PGI group where many oncologists actually appreciated your case report.
Hopefully we can send him to them and let's hope this is a treatable lymphoma
[11/25/2022, 6:07 PM] PaJR Jarvis 2020
:
Updating sir
[11/25/2022, 6:21 PM] :PaJR Jarvis 2020
[11/25/2022, 6:29 PM] Rakesh Biswas:
You need to upload the HRCT images showing the findings of "ground glass opacities and Halo sign"
Also you should have described the BAL report below the sentence where you said we did the BAL because of halo sign.
Also why haven't you mentioned the most important part of the BAL report that is showing positive for malignancy.
Please update the above and let me know asap
[11/26/2022, 9:46 AM] Rakesh Biswas: The statement in this report appears grossly contradictory to your current understanding? @PaJR Jarvis 2020
[11/26/2022, 9:52 AM] PaJR Jarvis 2020
: Updated sir yesterday
Will edit that part
[11/26/2022, 10:07 AM] Rakesh Biswas: What does it mean?
Does he have malignancy in his BAL fluid cytology or not?
[11/26/2022, 10:09 AM] : PaJR Jarvis 2020
Yes sir
Cytology is showing for malignancy
[11/26/2022, 10:11 AM] Rakesh Biswas: Am I not right about our need to have more training in reading and writing?
[11/26/2022, 10:13 AM] PaJR Jarvis 2020
: Yes sir
My mistake
[11/26/2022, 10:13 AM] Rakesh Biswas: Our mistake
[11/26/2022, 10:15 AM] Rakesh Biswas: When you do something good for the patient , it will be your own reward.
Mistakes are our collective responsibility 👍
[11/26/2022, 11:09 AM] PaJR Jarvis 2020
: Sir
I have talked to brig sir
They want to go for Inguinal lymph node biopsy
And also want to get a laryngoscope
Tumor board meeting might be on Wednesday itseems and
Probably tentative date for splenectomy is on Thursday sir.
[11/26/2022, 11:15 AM] Rakesh Biswas: Review the literature and share if splenectomy is advisable in lymphoma
[11/26/2022, 12:12 PM] Rakesh Biswas: Ours is not a primary splenic lymphoma.
At the moment the pathology appears to be in the lung and lymph nodes
[11/26/2022, 12:24 PM] Rakesh Biswas: Another reading (or texting) mistake of "ours" @PaJR Jarvis 2020
@ PaJR Jarvis 2019
Thyroid is not showing metastasis?
Did you review what is Thyroid Bethesda Category 2?
[11/26/2022, 12:25 PM] : PaJR Jarvis 2020
Yes sir
It is benign condition
[11/26/2022, 12:27 PM] PaJR Jarvis 2020
: I have mentioned thyroid is Bethesda’s 2 sir
[11/26/2022, 12:28 PM] Rakesh Biswas: 👆is this text right?
[11/26/2022, 12:29 PM]: PaJR Jarvis 2020
It was a text message mistake sir
I have mentioned again tagging that that it’s showing Bethesda 2
[11/26/2022, 12:30 PM] Rakesh Biswas: If text messages are meant for communication then they can also communicate mistakes
[11/27/2022, 11:25 AM] PaJR Jarvis 2020
: He is having fever spikes since the procedure.
[11/27/2022, 11:28 AM] Rakesh Biswas: Sepsis.
Hope he's on febrile neutropenia regime?
Please share his current antibiotic treatment schedule
[11/27/2022, 11:52 AM] PaJR Jarvis 2020
: We are to start with
Piptaz or magnex forte ( cefaperazone and sulbactam ) from today sir
These are based on Micro mam’s inputs from CDM sir
[11/27/2022, 11:53 AM] Rakesh Biswas: Look up the current guidleines for managing febrile neutropenia sepsis. Share in Microbology group
[11/27/2022, 11:53 AM] PaJR Jarvis 2020
: On it sir
[11/27/2022, 6:13 PM] Rakesh Biswas: In the 53M you shared here it's unclear if this was a primary splenic lymphoma.
Our 14M is unlikely to be primary splenic lymphoma given that he has good demonstrable involvement in the lung
Again many unanswered general knowledge questions around the origin of lymphomas in various organs (primary) followed by spread to other organs (secondarily) and PNI connections to his autoimmune past and his lymphomatous present both possibly driven by PNI @PaJR Jarvis
Psychologist @Metapsychist Number 1 Kims 2015
[11/27/2022, 7:09 PM] : PaJR Jarvis 2020
Lymphnodes sir
Although bone marrow biopsy was not done I guess sir
[11/27/2022, 7:30 PM] Rakesh Biswas: Don't guess. That's not a good part of our training program.
Check the NIMs discharge summary. As far as I can recall they did do the bone marrow and found nothing other than bone marrow hyperplasia suggesting hypersplenism
[11/27/2022, 7:39 PM] PaJR Jarvis 2020
: Sir
I meant with the shared case report, bcz bone marrow biopsy was mentioned.
Our patient’s
Bone marrow and lymph node which was done on 23th Sept showed reactive marrow
Normocellular
Normal distribution of rbc and myeloid lineage
Megakaryocytes were found around 4-6 on HPF
[11/29/2022, 9:13 PM] Rakesh Biswas: 12-1 PM Dhanwantari hall
[11/29/2022, 9:13 PM] Raveen 2020 Pg Med KIMs: Okay sir
[11/30/2022, 2:51 PM] Rakesh Biswas: @PaJR Jarvis 2020
Please show the patient to the pulmonologist and get his note suggesting that there is no pulmonological contraindication to OT tomorrow
[12/1/2022, 8:07 AM] PaJR Jarvis 2020
: I had night duty sir
Today morning I have ICU duty
I have put vamsi as replacement in ICU for the OT time sir
[12/1/2022, 8:14 AM] Rakesh Biswas: Vamsi will be with us in exams.
Find someone else for ICU
[12/1/2022, 8:30 AM] PaJR Jarvis 2020
: Manasa is replacing this
[12/24/2022, 4:11 PM] Rakesh Biswas: @PaJR Jarvis 2020
Please share his liver biopsy report done from both places asap
[12/24/2022, 4:32 PM] PaJR Jarvis 2020
: Biopsy Reports have been uploaded sir
Trends of hemogram post surgery are to be updated
Will share after its done sir
[12/25/2022, 10:42 PM] PaJR Jarvis 2020
: They came for follow up 2-3 days back sir
Jan 2023 patient is presented in the annual CPD on clinical complexity :
[2/28, 12:12 PM] PaJR Jarvis 2020
Sir he came for endoscopy
Gastro sir is not doing endoscopy it seems sir
[2/28, 2:06 PM] Raveen 2020 Pg Med KIMs: Thank you for the fish fry !! 😋@patient 14M
[2/28, 3:44 PM] patient 14M : It's my pleasure sir TQ sir 🙏🙏🙏
[3/2, 10:41 AM] PaJR Jarvis 2020
: Normal CBC 3 month follow up post surgery. Freed from the pancytopenia of his hypersplenism
[3/2, 10:56 AM] Rakesh Biswas: Great outcome without a diagnosis yet. @PaJR Jarvis 2018 Diagnosing is overrated!! 🙂
[3/2, 5:00 PM] PaJR Jarvis 2018
: Haha. Possibly.
But I think we may not have the same joy had the outcomes been otherwise?
I think inspite of us not fully knowing the diagnosis, splenectomy was the right step for whatever that diagnosis was. Diagnosis here is simply hidden data.
[3/2, 5:05 PM] Rakesh Biswas: The current 22F patient may also need to be operated with hidden data
[3/2, 5:06 PM] PaJR Jarvis 2018
: Yes sir.
[3/2, 5:11 PM] Rakesh Biswas: 👆@PaJR Jarvis 2020
It's not there in his case report. Please check if the sequence of information and investigations are updated there
[3/2, 7:24 PM] PaJR Jarvis 2020
: I updated in the blog sir
Both the outside liver biopsy
s/o NCPF
And our college
[3/2, 7:30 PM] Rakesh Biswas: That outside liver biopsy report was from?
We should place the different liver biopsy reports side by side in one image
[3/2, 7:30 PM] PaJR Jarvis 2020
: Thyrocare sir
Lab in Nalgonda
@PaJR Jarvis 2020c
Had also made a case report link on this patient. Can it be shared here?
[3/2, 7:31 PM] [3/2, 7:30 PM] PaJR Jarvis 2020
IF SPLEEN COULD SPEAK 😂
Some fun learning😄
Hope you guys like it!
[3/2, 7:37 PM] Rakesh Biswas: Thanks
I just checked your case report link and the last para of the case report is
Quote
20 days back he had history of pain abdomen and was admitted in the hospital in their vicinity and was advised splenectomy.
Is there any other link to his updated case report?
[3/2, 7:39 PM] Rakesh Biswas: Please update his case report with all the data in sequence including all images for example CT abdomen etc showing his massive spleen, his post operative spleen specimen etc
Also paste all the PaJR conversations since the PaJR began into a case report header "Discussion CDSS (conversational decision support system)
[3/2, 7:40 PM] Rakesh Biswas: Including histopathological images
[3/2, 7:27 PM] PaJR Jarvis 2018
: If indeed even our 22/F does have NCPF or BCS or Veno occlusive disease, will be interested in publishing a case series on their journies.
[5/5, 7:48 PM] PaJR Jarvis 2018
: @PaJR Jarvis 2020
when you find time, can you please update the case log and follow up. Only when you find time.
[5/5, 7:48 PM] PaJR Jarvis 2018
: Sharing it with some students here and they may not get the full picture when they see the blog.
[5/5, 7:56 PM] PaJR Jarvis 2018
: Okay sir
[5/14, 6:24 AM]PaJR Jarvis 2020
: 6 months follow up
[5/14, 6:57 AM] Rakesh Biswas: Current diagnosis?
[5/14, 7:53 AM] PaJR Jarvis 2020
: Sir am still skeptical about
Storage disorders
Am still going for a autoimmune disorders
I was reviewing yesterday night
Regarding
Can this be Primary immune regulatory Disorders ?? Or immunodeficiency ( Immune electrophoresis met a dead end for this )
Autoimmune cytopenias
Immunoglobulin class shifts
He is having eosinophilia too
Last time too
?Autoimmune hemolytic anemia
?Auto immune neutropenia
?Autoimmune thyroiditis ( fitting for NCPF top )
?Lymphoprolifetation
He still has lymphadenopathy
[5/14, 8:08 AM] PaJR Jarvis 2020
: His
Immunoelectrophoresis was showing
High levels of IgM
[5/14, 9:02 AM] PaJR Jarvis 2020
: Autoimmune lymphoproliferative syndrome (ALPS) is characterized by immune dysregulation due to a defect in lymphocyte apoptosis. The clinical manifestations may be noted in multiple family members and include lymphadenopathy, splenomegaly, increased risk of lymphoma and autoimmune disease, which typically involve hematopoietic cell lines manifesting as multilineage cytopenias.
The inherited genetic defect of many ALPS patients has involved (FAS) pathway signaling proteins, but there remain those patients who carry undefined genetic defects. Despite ALPS having historically been considered a primary immune defect presenting in early childhood, adult onset presentation is increasingly becoming recognized, and more so in genetically undefined patients and those with somatic FAS mutations.
The most common laboratory abnormalities found are cytopenias due to autoimmune destruction or splenic sequestration. Conversely, eosinophilia and monocytosis may also be associated findings[13]. Autoantibodies may be present and include positive Coomb’s direct antiglobulin test, rheumatoid factor (RF), or anti-nuclear antigen (ANA). Hypergammaglobulinemia is also frequently present[12]. Serum IL-10, soluble FAS ligand, and vitamin B12 are commonly elevated in ALPS patients with FAS mutations and can be useful biomarkers[14, 15] for these patients, but may not be abnormal in those with unidentified genetic mutations. Although not commercially available, flow cytometry of the blood for increased number of DNTs can be performed and is pathognomonic of ALPS
[5/14, 9:03 AM] :PaJR Jarvis 2020
[5/14, 9:03 AM] PaJR Jarvis 2020
: He is also fitting in ALPS 😅😅
[5/14, 9:03 AM] Rakesh Biswas: First tell us the morphologic diagnosis
[5/14, 9:06 AM] Rakesh Biswas: If you start with an infinite etiological approach every patient will fit into everything.
That's the reason history and clinical examination localization is the first step
What's the differential for a massive splenomegaly with dilated portal venous system?
[5/14, 9:13 AM] Rakesh Biswas: Where are the post operative images of his spleen sample and liver and spleen histopathology?
[5/14, 9:15 AM] Rakesh Biswas: It's the other way round?
Autoimmunity can produce secondary CVID?
[5/14, 11:03 AM] PaJR Jarvis 2020
: Functional - Mass per abdomen with recurrent vomiting
Recurrent respiratory tract infections
Anatomical - Portal vein
Pathological - portal vein fibrosis ( non cirrhotic portal vein fibrosis )
Etiological - immune mediated
Lymphoproliferative
Autoimmune spectrum
[5/14, 11:05 AM] Rakesh Biswas: Also add the legends to these images and you can borrow from the original pathology reports acknowledging the original pathologist's reports.
Take @PaJR Jarvis 2018
and an interested pathology faculty as coauthors if you want to report this in BMJ after your exams
[5/14, 11:15 AM] PaJR Jarvis 2020
: Absolutely sir 👍🏽
[5/14, 12:04 PM] PaJR Jarvis 2020
: Sir
Once review this article sir
ALPS presents in childhood with lymphadenopathy, hypersplenism, and multilineage cytopenias. Many patients experience a lessening of symptoms in adulthood, most likely due to age-associated immune modulation. ALPS due to germline FAS mutations is inherited in an autosomal dominant manner (2, 3), however somatic FAS mutations limited to circulating lymphocyte subsets leading to clinical manifestations of ALPS have also been reported over the years
Over many years of follow-up, the general morbidity attributable to ALPS includes the frequent need for splenectomy and the risks of overwhelming post-splenectomy infection (OPSI) leading to sepsis, recurrent and chronic multilineage cytopenias, and development of lymphomas
ALPS patients have been noted to have a unique IgM-mediated immune surveillance defect following exposure to pneumococci (7–9). Among the 66 splenectomized patients in our recently published cohort, 41% (n = 27) had multiple episodes of pneumococcal sepsis, and 6 of them died. The likelihood of cytopenia relapse after splenectomy was 30% by 4 years and exceeded 70% by 20 years thus underscoring the futility of splenectomy itself as an intervention.
We recently summarized our experience related to cytopenias in 150 patients with ALPS-FAS with a median follow-up of 13.5 years (8). Recurrent multilineage cytopenias were common, seen in two-thirds (104/150) of them. Their median age of initial presentation was 5.6 years with a range of age of disease onset from 1 to 53 years. There were no gender differences. Single lineage, bilineage, and trilineage cytopenias were seen in 21, 23, and 25% of them, respectively
Coomb’s DAT positive without overt hemolytic anemia
[5/14, 12:21 PM]PaJR Jarvis 2020
: He was having generalised lymphadenopathy right from the beginning sir
We did once for cervical
And one in inguinal region
One cervical biopsy in NIMS
Post operatively
Mesenteric sir
[5/14, 12:21 PM] Rakesh Biswas: What's the histopathology of that?
[5/14, 12:22 PM] PaJR Jarvis 2020
: They gave reactive for Mesenteric
Rosai dorfman for
Cervical and inguinal 😅
[5/14, 12:25 PM] Rakesh Biswas: Are all these sequence of events for this patient arranged in an appreciable sequence in your patient?
[5/14, 12:27 PM] PaJR Jarvis 2020
: Yes sir
[5/27, 2:56 PM] PaJR Jarvis 2020
: Update from the father
He had a history of fall
Causing left forearm both bone fractures
Based on the fathers note
He had ? boils( ?eating a lot of mangoes)
5/27, 4:15 PM] Rakesh Biswas: How bad was the fall? Or was this a pathological fracture?
[5/27, 5:55 PM] PaJR Jarvis 2018
: I have asked for the x ray
Yet to be shared sir
[5/28, 12:44 AM] Rakesh Biswas: Thanks
After the above entry in May 2023 the next update in November 2023 is the father's phone call informing about his son's death
