David Steensma, MD Profile picture
Dec 21, 2022 13 tweets 8 min read Read on X
I’ve never posted a @tiktok_us link before, but this is a rare opportunity to observe one of the most poorly understood disorders in #hematology: Gardner-Diamond syndrome. This young woman's skin lesions first appear about 40 seconds in./1 #MedTwitter tiktok.com/@nancy.xoxx/vi…
The syndrome was described by Drs. Frank Gardner (1919-2013) and Louis K. Diamond (1902-1999) in Boston in 1955, in @BloodJournal. They reported 4 cases, all women, who had a peculiar form of bruising on face or extremities but had no other bleeding & normal coagulation tests./2
The key finding in Gardner-Diamond syndrome: unexplained painful bruises, most commonly on extremities or face, often during times of stress. The pathophysiology is unclear, as described below. Many patients have been dismissed by physicians as having a fictitious disorder./3 Facial ecchymoses on Nancy Morel's TikTok
We’ve discussed Louis Diamond before, as he gave his name to a number of hematological disorders. Frank Gardner’s primary contribution to hematology was recognizing that platelets could be stored for 4-5 days at room temperature, important for blood banks: ashpublications.org/thehematologis…
Somewhat confusingly, Gardner & Diamond used the term “autoerythrocyte sensitiziation” because they took patients’ own RBCs & injected them into the skin, which caused ecchymosis, but didn’t see that with donor or animal blood. This finding has not been consistently replicated./5
In 1962, David Agle and Oscar Ratnoff (1916-2008) @cwru published a paper in @JAMA_current emphasizing the psychiatric component of the disorder, including a high frequency of concomitant conversion disorder, and sometimes an abuse history: jamanetwork.com/journals/jamai…
Ratnoff, who specialized in thrombosis/hemostasis and described factor XII, almost 3 decades later (1989) published a serious of 71 patients with what by then was also known as "psychogenic purpura". He noted surgery or an injury preceded onset of symptoms in many cases./7
The @MayoClinic coag group, including the late Bill Nichols, in 2019 published a series of 76 cases seen over a 40 year period. Prior surgery or injury were common. With psychological counseling/medications, ~1/2 of patients had resolution of symptoms. amjmedsci.org/article/S0002-…
Because of the psychiatric component, some doctors are dismissive of psychogenic purpura. In fact a dermatologist from California posted on Ms. Morel's TikTok account after she had shared images of her rash, suggesting that the video was faked. This post was removed. /9
Some have suggested Gardner-Diamond as a possible mechanism for stigmata, in which people develop bruises mimicking Jesus Christ's crucifixion wounds during periods of intense religious or mystical experience, as seen on this mystic's hands & described in a 1980 case report./10
When coagulation tests have been performed in "GDS", they are typically normal. In fact the diagnostic approach is to rule out everything else, especially von Willebrand disease & other inherited and acquired hemorrhagic diatheses like DIC. There's no specific diagnostic test./11
@ben_geisler & Nicholas Kontos @MGHMedicine wrote a nice @UpToDate article on GDS/psychogenic purpura. They summarize several mechanisms by which stress *might* cause minor hemostatic alterations, as summarized in the attached image. The full article is well worth reading./12
The key is to be supportive of the patient and non-judgmental, take their concerns seriously, carefully rule out other coagulation and dermatological disorders, and provide appropriate emotional and psychiatric support. Sometimes it will resolve. /13End

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More from @DavidSteensma

Nov 29, 2023
Did you ever wonder why our marrow is located inside of our *bones*, #MedTwitter? There’s no a priori anatomical reason it should be sited there. Blood cells could form in our spleens & livers, as they do during our fetal lives; or elsewhere, as in some animals. Let’s discuss! /1
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After all, zebrafish get along just fine making blood cells in their “kidney marrow”, keeping @leonard_zon's lab running. Drosophila make blood-like cells in abdominal hubs, and frogs… frogs mix it up. Jeremiah Bullfrog & frog cousins make blood in kidneys, marrow, liver, etc./2


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Hematopoietic stem cells (HSCs) are like seafarers or long-haul truckers: they have a tough, unforgiving job requiring long hours. They must generate >2×10¹¹ blood cells daily, over the course of an organismal lifespan that might span >100 years. (HSC time image: @Goodell_Lab)/3
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Read 19 tweets
Sep 6, 2023
I was reviewing marrow failure history in preparation for an upcoming @aamdsif conference, and I’m completely awed by the observational skills of young physician Paul Strübing in Greifswald, Germany in 1882 describing the first clear case of paroxysmal nocturnal hemoglobinuria./1 Image
This was the only photo I could find of Strübing, from over 20 years later (circa 1905). At the time of publication of his prescient 1882 paper, Strübing was 29 years old and had graduated from medical school 6 years earlier. /2 Image
As William Crosby @TuftsMedSchool wrote in 1951 @BloodJournal, “Strübing’s paper on [PNH] might well be regarded as a classic of clinical investigation. He recognized clearly that the disease was a new entity and characterized it by a careful description of his patient."/3

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Read 14 tweets
May 2, 2023
Today marks the end of an era: publication of the final philatelic vignette (authors.elsevier.com/a/1g-uM5qq8Xpp3) co-authored by Robert Kyle @MayoClinic. Bob turned 95 this year and decided to step down from helping prepare these. He published his first vignette @JAMA_current back in 1969!/1 Image
This longstanding series focused on medical & scientific-themed postage stamps began in 1961. At the 109th Annual Meeting of @AmerMedicalAssn in the then-new convention center in Miami Beach, Florida in June 1960, John Mirt (1897-1968) discussed “Medical Pathfinders on Stamps”./2 ImageImage
At the time, Mr. Mirt – a devoted Freemason - worked in press relations for the AMA in Chicago. After the meeting, he was asked by then-editor of @JAMA_current John Talbott (1902-1990) to start composing short articles about physicians & scientists featured on postage stamps./3 ImageImage
Read 21 tweets
Mar 27, 2023
@OpenAI #ChatGPT4 "hallucinations" remind me of a now-amusing event from way back in Grade 3 when I was 7, involving the last time I knowingly confabulated. I was assigned to write a report about airplanes.✈️ One thing I already knew about airplanes: they were expensive! (1/12) Stock photo of a blond girl...
According to my parents, the high cost of airplane tickets was why our family always drove the tedious 800+ miles from our NYC suburban home to see relatives in Michigan at Christmas🎄If 4 airplane *tickets* were expensive, I reasoned an *entire airplane* must be hugely costly. Cool airplane origami made ...
I found lots of good airplane info, which went into that report. But couldn't find ANYTHING about cost. I looked everywhere a kid could look in the 1970s: encyclopedias, school books, library books. Nothing. Desperate, I made up a plausible number 😲 and added it to the report. 1970s World Book.  I used o...
Read 14 tweets
Nov 23, 2022
Among the many things I am #thankful for: recent progress in hematologic malignancies. When I started my career - not that long ago! - standard therapy for myeloma was melphalan & prednisone or VMP, chlorambucil for CLL, CHOP (without R) for NHL, epoetin & transfusions for MDS./1
CML was treated with Hydrea, busulfan, or interferon & Ara-C,and the big debate was transplant timing. Most patients didn't have an allo transplant donor & the age cutoff was 50-55. Karyotyping was inconsistently done even in AML/MDS; FISH was new; single gene testing was rare./2
No one knew about JAK2 mutations, let alone envisioning specific JAK2 inhibitors. There were elderly patients with polycythemia we treated with radiophosphorous. Many clinical trials were small IITs. The most "exciting" progress was in hairy cell leukemia: pentostatin & 2-CDA./3
Read 6 tweets
Oct 30, 2022
A medical textbook nostalgia thread! I dug up my @UChicagoMed transcript & recalled what books we used for each class. Buying books online was rare back then, so most came from the University bookstore at 58th & S Ellis, or the claustrophobic but amazing @SeminaryCoop on 57th./1 ImageImageImage
Year 1, Term 1 (Autumn): Netter’s Atlas of Human Anatomy (1989 edition), Wheater’s Functional Histology (1987), Moore’s Developing Human: Clinically Oriented Embryology (1988), Stryer’s Biochemistry (1990). (If you had another biochem text from undergrad, you could use that.)/2 ImageImageImageImage
One weird fact about U of C medical school: back then med student skeletal anatomy was taught by paleontologists, including @NatGeo Explorer-in-Residence Paul Sereno (here's his Facebook image). So we learned not just about human anatomy, but how humans differ from dinosaurs.🦖🦕 Image
Read 19 tweets

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