Thread by @RosenelliEM

Time for some #SpacedRepetition: @CPSolvers

Chat recap of the #ClinicalPearls #VirtualMorningReport

June 10th Day 69: clinicalproblemsolving.com/morning-report…

w/ @arturohdez_1 @RWBonner91 @PaulKunnath
@DxRxEdu @rabihmgeha

Teaching points clinicalproblemsolving.com/wp-content/upl… by @sukritibanthiya

Before we begin let’s make sure we focus and highlight some things on #racialbias

A great article on this topic was shared. Racial bias in pain assessment & treatment recommendations, and false beliefs about biological differences between blacks & whites
ncbi.nlm.nih.gov/pmc/articles/P…

Let’s start with a problem representation:

A 28 y/o M w/ a h/o hemophilia A presents with lightheadedness, dyspnea, LE bruising 1 week prior and found to have an ⬆️ bill, hypo-proliferative anemia (7.3 g/dL), ⬆️ PTT, low B12 & folate without evidence of bleeding on imaging

My initial thought to focus around the ddx of acute/subacute hemoglobin drop given he had a normal CBC 6 months prior.

The highest concern would be for bleeding.

Any history things that are helpful other than delayed healing vs spontaneous brushing?

DOE and lightheadedness likely related to anemia here, given no other signal for cardiopulmonary disease
who has the coag cascade memorized?

Intrinsic vs extrinsic pathways
For Intrinsic think PTT (play table tennis) like you play that (inside) Factor 12, 11, 9, 8

vs Extrinsic for PT(play tennis) like you play that outside (extrinsic) Factor 7

I like the mixing study idea. I wonder if he has acquired a

Factor 8 antibody given his likely history of frequent transfusion

Part of well-made management scripts - initial resuscitation treatment interwoven with diagnostic evaluation

most hemophiliacs aren't symptomatic until like <5% of normal F8 levels

Bleeding from clotting factors def into deep tissues …

From thrombocytopenia/platelet defects is mucocutaneous

The usual bleeding ROS answers: hemoptysis, nose bleeds, gum bleeding, melena/hematochezia, etc...
lower extremity bruises are usually due to platelets low/platelet dysfunction… But coag fact def does it cause bruising?

Seemingly pointing away from cardiopulmonary cause but if time course was longer, could consider untreated anemia leading to high output CHF

Anyone else thinking Diffuse alveolar hemorrhage (DAH) potentially causing his severe anemia and SOB
repeated

DAH in a patient with hemophilia B, pubmed.ncbi.nlm.nih.gov/28202865/

Why the jaundice? Increased hemolysis, Now we need CBC + hemolysis labs + smear

autoimmune vs mechanical hemolysis?

Something like cryoglobulinemia could support the MAHA + vasculitis like the picture.

You can also get an increase in bili from resorption from ecchymosis.

Not sure if that alone could cause the icterus?

if you have a proliferative anemia, think bleeding or hemolysis

Viruses with BM involvement- parovovirus B19, dengue, hepatitis, Epstein-Barr virus, CMV, HIV

Looks like he has a Hypoproliferative anemia with a <2 RPI, so unlikely hemolysis based off these labs

Also, haptoglobin isn’t low, LDH not elevated

Cbc 6 mo ago: hub 15.8, WBC 5.8, plt 275 hmmmm

Any corkscrew hair or perifollicular hemorrhage?
bleed - leukopenia - hypoproliferative anemia. any link?

Lymphoma infiltrating marrow can cause cytopenias and paraneoplastic fact 8 inhibitors

thinking of an EBV-driven B cell lymphoma —> acquired factor VIII inhibitor

immunological alterations induced by continuous antigenic stimulate derived from plasma concentrate could cause leukopenia

HLH explains bicytopenia but most of the time you have fevers, only 50% of HLH is due to cancer

Glanzmann is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet aggregation....can look for GP IIB/IIIA activity, ncbi.nlm.nih.gov/pmc/articles/P…

SLE can cause ab's against factor VIII, it also can cause

Antibody's against RBC's causing a hemolytic anemia
SLE also can cause leukopenia cuz of Ab's

acquired vWF deficiency can manifest at any time.. even aspirin use can trigger subclinical vWF issues

B12 and folate are super needed for bone marrow production of cells.

So maybe his low levels are the explanation?
fascinating that vitamin deficiency would present so acutely so likely suggests chronic, progressive process with tip over

There are causes of ‘Pseudo-petechiae’
vitamin c can also cause perifollocular and mucosal bleeding

It's rare to see b12 deficiency because the human body stores take months-years to deplete.

Folate and B12 are absorbed in the terminal ileum if I remember correctly, so maybe there's an issue there.

An MMA level would be great

If MMA & homocysteine are⬆️& the vitamin B12 level is mildly🔽then an early/mild B12 deficiency may be present.

This may indicate a 🔽in available B12 at the tissue level. If only homocysteine level is ⬆️& not MMA, then the person may have a folate def ncbi.nlm.nih.gov/pmc/articles/P…

B12 absorbed in ileum and folate in jejunum
a chronic hemolytic anemia can deplete b12 and folate stores, what was his diet?

Usually to get scurvy you have to have very, very low fruit and veggie intake.

it takes months for folate def and 4-5 years to develop b12 def

What is a pirates favorite letter? 10 of them...I, I, R, and the seven Cs

Recent image in clinical medicine case on scurvy in NEJM: nejm.org/doi/full/10.10…

There were a lot of twists and turns (corkscrew hairs) in this case, lol

Think factor deficiencies/quantitive plt problems/qualitative platelet problems/vascular wall integrity (vasculitis/scurvy, etc)

Some Still see scurvy in populations with food insecurity
food faddists or malabsorption. this is where we see scurvy nowadays
ncbi.nlm.nih.gov/pmc/articles/P…

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