I****a
发帖数: 407 | 1 33 y/o white male with history of type I DM on insulin, PVD, s/p fem/pop
bypass 2 years ago who presented to local ER with bloody diarrhea, nausea,
vomiting 2 days after he visited a casino and was reported eating a raw beef
hamburger. He quickly developed AMS and was intubated for airway protection.
His admitting labs were noted having a marked normocytic anemia and his
blood smear was noted having significant agglutination. He has 3+ warm
antibody presence on DAT. His hgb was in low 4s and he was transfused
urgently with uncross matched blood. He also developed renal failure since
day 2 of admission.
ROS: all (-) except I mentioned above.
SH: (-) X3, construction worker, single, sexually inactive in the last 3
months
FH: non-contributory
What are you differentials? What are the labs you want and what would you do? |
b******a
发帖数: 704 | 2 More PE? Thank you for sharing the case!
beef
protection.
【在 I****a 的大作中提到】
: 33 y/o white male with history of type I DM on insulin, PVD, s/p fem/pop
: bypass 2 years ago who presented to local ER with bloody diarrhea, nausea,
: vomiting 2 days after he visited a casino and was reported eating a raw beef
: hamburger. He quickly developed AMS and was intubated for airway protection.
: His admitting labs were noted having a marked normocytic anemia and his
: blood smear was noted having significant agglutination. He has 3+ warm
: antibody presence on DAT. His hgb was in low 4s and he was transfused
: urgently with uncross matched blood. He also developed renal failure since
: day 2 of admission.
: ROS: all (-) except I mentioned above.
|
I****a
发帖数: 407 | 3 His exam was not remarkable at the time of admission. |
A*******s
发帖数: 9638 | 4 Autoimmune Hemolytic Anemia。
Did he take any abtibotics for the diarrhea? |
b******a
发帖数: 704 | 5 any medication history? any current treatment for his PVD?
Sorry to ask so much information.
My first impression is HUS, triggered by E. coli, Camplyobacter etc.
DDX: TTP, HSP, Sepsis/DIC, Intussusception.
First ask for CBC with diff, Peripheral blood smear, BMP, BUN/cr, stool
bacteria culture.
【在 I****a 的大作中提到】
: His exam was not remarkable at the time of admission.
|
s**********t
发帖数: 217 | 6 Escherichia coli O157:H7.
With HUS.
beef
protection.
【在 I****a 的大作中提到】
: 33 y/o white male with history of type I DM on insulin, PVD, s/p fem/pop
: bypass 2 years ago who presented to local ER with bloody diarrhea, nausea,
: vomiting 2 days after he visited a casino and was reported eating a raw beef
: hamburger. He quickly developed AMS and was intubated for airway protection.
: His admitting labs were noted having a marked normocytic anemia and his
: blood smear was noted having significant agglutination. He has 3+ warm
: antibody presence on DAT. His hgb was in low 4s and he was transfused
: urgently with uncross matched blood. He also developed renal failure since
: day 2 of admission.
: ROS: all (-) except I mentioned above.
|
s**********t
发帖数: 217 | 7 Escherichia coli O157:H7.
With HUS.
beef
protection.
【在 I****a 的大作中提到】
: 33 y/o white male with history of type I DM on insulin, PVD, s/p fem/pop
: bypass 2 years ago who presented to local ER with bloody diarrhea, nausea,
: vomiting 2 days after he visited a casino and was reported eating a raw beef
: hamburger. He quickly developed AMS and was intubated for airway protection.
: His admitting labs were noted having a marked normocytic anemia and his
: blood smear was noted having significant agglutination. He has 3+ warm
: antibody presence on DAT. His hgb was in low 4s and he was transfused
: urgently with uncross matched blood. He also developed renal failure since
: day 2 of admission.
: ROS: all (-) except I mentioned above.
|
I****a
发帖数: 407 | 8 Yes he has AIHA. He was not taking any abx.
【在 A*******s 的大作中提到】
: Autoimmune Hemolytic Anemia。
: Did he take any abtibotics for the diarrhea?
|
d**o
发帖数: 618 | 9 楼上大牛都分析得相当透彻，佩服。
我弱弱的补充一句：HUS can also be caused by Shigella dysenteriae serotype 1,
which can also be transmitted by raw meat.
再弱弱的问一句：Why didn't Icetea mention or describe fever? |
d**o
发帖数: 618 | 10 Well now I'm confused, hehe. Is this antiphospholipid syndrome? Is the hamburger just a red herring? Is the agglutination the key?
【在 I****a 的大作中提到】
: Yes he has AIHA. He was not taking any abx.
|
|
|
I****a
发帖数: 407 | 11 Good thoughts so far. I remember he was taking ASA prior to admission.
I will fast forward a little bit.
All the cultures you can think of are all negative.
His coags were cold normal at the time of presentation and several days
later. His smear showed some spherocytes which is consistent with hemolysis.
There was no schistocytosis.
His white cell were slightly elevated with slight neutrophilia.
His platelet count was normal but by day 3-5 into the admission, it dropped
to 40-50K.
His liver function tests were normal at the time of presentation but AST/ALT
went to lower 1000s range 5 days later.
He had LP which showed high elevated protein 289, there are no elevated WBC
or RBC. Glucose was in normal range. Viral/bacterial/fungal cultures/pcr
were all negative.
His initial CT scan of abdomen was normal but 5 days later showed a small
splenic infarction with mild-moderate splenomegaly.
A++ was correct he had warm ab hemolysis however it does not explain all his
problems. He was started on high dose steroid.
He also had bone marrow biopsy on day 5 when thrombocytopenia developed
however it was pretty much normal looking marrow.
I will throw in some more spice:
1. His ESR was 130
2. By day 7, he became aneuric and he was started on dialysis, his UA showed
large blood but only couple RBCs.
3. By day 7-10, he became pancytopenia with total white cell 1K then dropped
to 0.5K, a repeat bone marrow biopsy was performed which showed:
complete bone marrow necrosis.
What is going on ? What is your differential now?
【在 b******a 的大作中提到】
: any medication history? any current treatment for his PVD?
: Sorry to ask so much information.
: My first impression is HUS, triggered by E. coli, Camplyobacter etc.
: DDX: TTP, HSP, Sepsis/DIC, Intussusception.
: First ask for CBC with diff, Peripheral blood smear, BMP, BUN/cr, stool
: bacteria culture.
|
I****a
发帖数: 407 | 12 I like your antiphospholipid syndrome hypothesis.
ANA were sent which were negative.
His aptt was initially normal but later he had mild coagulopathy however
lupus anticoagulant was not present. anti-cardiolipin antibody and b2
glycoprotein ab were not present either.
hamburger just a red herring? Is the agglutination the key?
【在 d**o 的大作中提到】
: Well now I'm confused, hehe. Is this antiphospholipid syndrome? Is the hamburger just a red herring? Is the agglutination the key?
|
I****a
发帖数: 407 | 13 He did not have fever when he firstly presented but he had some intermittent
low grade fever during the hospitalization, which has been over 6 weeks.
1,
【在 d**o 的大作中提到】
: 楼上大牛都分析得相当透彻，佩服。
: 我弱弱的补充一句：HUS can also be caused by Shigella dysenteriae serotype 1,
: which can also be transmitted by raw meat.
: 再弱弱的问一句：Why didn't Icetea mention or describe fever?
|
c****e
发帖数: 188 | 14 Looks like TTP to me...Does he have any stroke? any head CT or MRI?
last week I did OB in a hospital and saw a similar case like that, so TTP came into my mind immediately...
hemolysis.
dropped
ALT
【在 I****a 的大作中提到】
: Good thoughts so far. I remember he was taking ASA prior to admission.
: I will fast forward a little bit.
: All the cultures you can think of are all negative.
: His coags were cold normal at the time of presentation and several days
: later. His smear showed some spherocytes which is consistent with hemolysis.
: There was no schistocytosis.
: His white cell were slightly elevated with slight neutrophilia.
: His platelet count was normal but by day 3-5 into the admission, it dropped
: to 40-50K.
: His liver function tests were normal at the time of presentation but AST/ALT
|
I****a
发帖数: 407 | 15 I agree with you TTP is in differential. For educational purpose, the
classical pentad for TTP are: thrombocytopenia, mcroangiopathic hemolytic
anemia with schistocytosis, fever, renal failure and CNS findings. This
patient has 4 out 5. He has no schistocytosis on multiple blood smears. Can
you make diagnosis of TTP?
came into my mind immediately...
【在 c****e 的大作中提到】
: Looks like TTP to me...Does he have any stroke? any head CT or MRI?
: last week I did OB in a hospital and saw a similar case like that, so TTP came into my mind immediately...
:
: hemolysis.
: dropped
: ALT
|
A*******s
发帖数: 9638 | 16 What is Vitamin B12 level?
How about Glutathione Reductase Deficiency? |
I****a
发帖数: 407 | 17 B12, folate level were normal. Urine/blood toxicology were non revealing.
I have never heard of glutathione reductase deficiency. Can it link
everything together?
【在 A*******s 的大作中提到】
: What is Vitamin B12 level?
: How about Glutathione Reductase Deficiency?
|
c****e
发帖数: 188 | 18 google and found this......
Thrombotic Thrombocytopenic Purpura Without Schistocytes on the Peripheral
Blood Smear
http://journals.lww.com/smajournalonline/fulltext/2005/03000/th
Can
【在 I****a 的大作中提到】
: I agree with you TTP is in differential. For educational purpose, the
: classical pentad for TTP are: thrombocytopenia, mcroangiopathic hemolytic
: anemia with schistocytosis, fever, renal failure and CNS findings. This
: patient has 4 out 5. He has no schistocytosis on multiple blood smears. Can
: you make diagnosis of TTP?
:
: came into my mind immediately...
|
I****a
发帖数: 407 | 19 For the benefit of doubt, he was plasma exchanged to treat presumptive TTP.
The ADAMTS13 level was 42% before the first exchange. He also got a 1-2
doses of IVIG prior to plasma exchange.
The ADAMTS13 level of the classic and severe TTP causing this much damage
usually is very low, so 42% is not that impressive.
Finally he was exubated but he remained dialysis dependent. He is
pancytopenic, he is neutropenic with ANC of 0.2. He has more bloody diarrhea
, his platelet dropped to low teens and he required daily blood product.
GI was reluctant to scope him. He is not candidate for kidney biopsy due to
severe thrombocytopenia.
His mental status came back.
His ferritin was above 50,000. His triglyceride was couple thousands.
Come on guys!
Can
【在 I****a 的大作中提到】
: I agree with you TTP is in differential. For educational purpose, the
: classical pentad for TTP are: thrombocytopenia, mcroangiopathic hemolytic
: anemia with schistocytosis, fever, renal failure and CNS findings. This
: patient has 4 out 5. He has no schistocytosis on multiple blood smears. Can
: you make diagnosis of TTP?
:
: came into my mind immediately...
|
I****a
发帖数: 407 | 20 Yes, it could happen as you pointed out therefore patient was empirically
exchanged.
【在 c****e 的大作中提到】
: google and found this......
: Thrombotic Thrombocytopenic Purpura Without Schistocytes on the Peripheral
: Blood Smear
: http://journals.lww.com/smajournalonline/fulltext/2005/03000/th
:
: Can
|
|
|
A*******s
发帖数: 9638 | 21 glutathione reductase deficiency can cause hemolytic anemia. There is also
a report regarding glutathione reductase deficiency induced hemolytic anemia
and pancytopenia. The triggers could be infection or meds.
Vit B2 is the treatment in this case.
http://www.nejm.org/doi/full/10.1056/NEJM197012032832304
【在 I****a 的大作中提到】
: B12, folate level were normal. Urine/blood toxicology were non revealing.
: I have never heard of glutathione reductase deficiency. Can it link
: everything together?
|
I****a
发帖数: 407 | 22 It is good to know this.
also
anemia
【在 A*******s 的大作中提到】
: glutathione reductase deficiency can cause hemolytic anemia. There is also
: a report regarding glutathione reductase deficiency induced hemolytic anemia
: and pancytopenia. The triggers could be infection or meds.
: Vit B2 is the treatment in this case.
: http://www.nejm.org/doi/full/10.1056/NEJM197012032832304
|
a********n
发帖数: 182 | |
a********n
发帖数: 182 | 24 他还有hypertriglyceridemia 引起的出血性胰腺炎，这个可引起他的消化道症状和多
器官功能衰竭。 |
I****a
发帖数: 407 | 25 Good job! That is the most likely diagnosis. Blow is the direct copy of
blood paper, How I treat hemophagocytic lymphohistiocytosis
1. Michael B. Jordan1,2,*,
2. Carl E. Allen3,*,
3. Sheila Weitzman4,
4. Alexandra H. Filipovich2, and
5. Kenneth L. McClain3
The diagnosis of HLH† may be established:
A. Molecular diagnosis consistent with HLH: pathologic mutations of PRF1,
UNC13D, Munc18-2, Rab27a, STX11, SH2D1A, or BIRC4
or
B. Five of the 8 criteria listed below are fulfilled:
1. Fever ≥ 38.5°C
2. Splenomegaly
3. Cytopenias (affecting at least 2 of 3
lineages in the peripheral blood)
Hemoglobin < 9 g/dL
(in infants < 4 weeks: hemoglobin < 10 g/dL)
Platelets < 100 ×
103/mL
Neutrophils < 1 ×
103/mL
4. Hypertriglyceridemia (fasting, > 265 mg/dL)
and/or hypofibrinogenemia (< 150 mg/dL)
5. Hemophagocytosis in bone marrow, spleen,
lymph nodes, or liver
6. Low or absent NK-cell activity
7. Ferritin > 500 ng/mL‡
8. Elevated sCD25 (α-chain of sIL-2 receptor)§
His natural killer cell function is absent. His sCD25 is elevated. So he has
7 out 8 from this list. We did not catch phagocytosis from 3 bone marrow
biopsies but it is probably not that important to make the diagnosis on this
case.
My hypothesis is that his immune system was marked dysregulated and
activated by the initial GI infectious agent, this led to what seems to be
autoimmunity with his autoimmune hemolysis, the cytotoxic T cell started
chewing up multiple organs. I believe this is the what it says secondary or
acquired HLH in contrast to hereditary ones that has specific gene mutation.
The treatment of the genetic ones are chemotherapy followed by stem cell
transplant. For my patient, we gave him steroid and later on with addition
of cyclosporin. He has recovered his counts, needing less blood product. His
kidney function is slowly improving.
Finally he requested his case to be written.
【在 a********n 的大作中提到】
: 好久不来，这里很热闹呵。
: Google了一下，是不是这个HLH？
: http://www.ncbi.nlm.nih.gov/pubmed/18085676
|
I****a
发帖数: 407 | 26 I strongly recommend to read this article by the most famous HLH experts.
http://bloodjournal.hematologylibrary.org/content/118/15/4041.f |
b******a
发帖数: 704 | 27 早应该知道冰瓜儿大夫的病例不会简单。 翻书，翻书。。。
难道是malignance 有关？
neoplasm TTP (diagnosed clinically)-->multiple organ vascular occlusion,
or acquired or 2nd HLH??? 要不怎么会要做肠镜排除肿瘤？
http://www.asco.org/ascov2/Meetings/Abstracts?&vmview=abst_deta
http://online.haematologica.org/e-cases/2002_01/01.htm
http://emedicine.medscape.com/article/986458-clinical
不可思议的是初期大部分正常，病程进展如此凶险，太吓人了。
dropped
ALT
He had LP which showed high elevated protein 289, there are no elevated WBC
or RBC. Glucose was in normal range. Viral/bacterial/fungal cultures/pcr
were all negative.
His initial CT scan of abdomen was normal but 5 days later showed a small
splenic infarction with mild-moderate splenomegaly.
A++ was correct he had warm ab hemolysis however it does not explain all his
problems. He was started on high dose steroid.
He also had bone marrow biopsy on day 5 when thrombocytopenia developed
however it was pretty much normal looking marrow.
I will throw in some more spice:
1. His ESR was 130
2. By day 7, he became aneuric and he was started on dialysis, his UA showed
large blood but only couple RBCs.
3. By day 7-10, he became pancytopenia with total white cell 1K then dropped
to 0.5K, a repeat bone marrow biopsy was performed which showed:
complete bone marrow necrosis.
What is going on ? What is your differential now?
【在 I****a 的大作中提到】
: I strongly recommend to read this article by the most famous HLH experts.
: http://bloodjournal.hematologylibrary.org/content/118/15/4041.f
|
I****a
发帖数: 407 | 28 There are still some stuff in his case that is never reported to be
associated with HLH such as complete bone marrow necrosis, a topic itself
worthy of discussion.
【在 b******a 的大作中提到】
: 早应该知道冰瓜儿大夫的病例不会简单。 翻书，翻书。。。
: 难道是malignance 有关？
: neoplasm TTP (diagnosed clinically)-->multiple organ vascular occlusion,
: or acquired or 2nd HLH??? 要不怎么会要做肠镜排除肿瘤？
: http://www.asco.org/ascov2/Meetings/Abstracts?&vmview=abst_deta
: http://online.haematologica.org/e-cases/2002_01/01.htm
: http://emedicine.medscape.com/article/986458-clinical
: 不可思议的是初期大部分正常，病程进展如此凶险，太吓人了。
:
: dropped
|
A*******s
发帖数: 9638 | 29 Did michael Jordan go to medical school after his NBA career? lol
Thanks Icetea and adropofsun, both of you will get Baozi.
【在 I****a 的大作中提到】
: Good job! That is the most likely diagnosis. Blow is the direct copy of
: blood paper, How I treat hemophagocytic lymphohistiocytosis
: 1. Michael B. Jordan1,2,*,
: 2. Carl E. Allen3,*,
: 3. Sheila Weitzman4,
: 4. Alexandra H. Filipovich2, and
: 5. Kenneth L. McClain3
: The diagnosis of HLH† may be established:
: A. Molecular diagnosis consistent with HLH: pathologic mutations of PRF1,
: UNC13D, Munc18-2, Rab27a, STX11, SH2D1A, or BIRC4
|
d**o
发帖数: 618 | 30 这个可以在好杂志上发个case report了吧，景仰！ |
|
|
A*******s
发帖数: 9638 | 31 No wonder I could not find any clue on this. lol
【在 I****a 的大作中提到】
: There are still some stuff in his case that is never reported to be
: associated with HLH such as complete bone marrow necrosis, a topic itself
: worthy of discussion.
|
I****a
发帖数: 407 | 32 LOL! and thanks for the BaoZi
【在 A*******s 的大作中提到】
: Did michael Jordan go to medical school after his NBA career? lol
: Thanks Icetea and adropofsun, both of you will get Baozi.
|
b******a
发帖数: 704 | 33 谢谢Icetea和adropofsun大夫。
这个得慢慢看看， 今天学习hematology，很受益。
【在 I****a 的大作中提到】
: I strongly recommend to read this article by the most famous HLH experts.
: http://bloodjournal.hematologylibrary.org/content/118/15/4041.f
|
a********n
发帖数: 182 | 34 Great feeling. I need to do a hemo/onco fellowship, not to be a PCP forever.
LOL. |
a********n
发帖数: 182 | 35 谢谢版大。
包子很香！
【在 A*******s 的大作中提到】
: Did michael Jordan go to medical school after his NBA career? lol
: Thanks Icetea and adropofsun, both of you will get Baozi.
|
k**o
发帖数: 18 | 36 it sounds like hemophagocytic syndrome with high ferritin and triglyceride.
It usually does not cause marrow necrosis. I've been thinking transfusion
associated graft versus host disease with marrow involvement. Mortality with
the latter is nearly 100%.
great case. |
k**o
发帖数: 18 | 37 hemophagocytic syndrome with high ferritin and triglyceride. It does not
usually cause marrow necrosis. I've been thinking of transfusion associated
graft vs host disease, but these pts ususally have extremely high mortality
rate
great case. |
w********e
发帖数: 762 | 38 you guys did nor do ferritin from beginning? since we had recently diagnosed
two HLH cases by earlier on. ferritin > 100,000 I think every new anemia pt
deserve a ferritin level
these two also marrow proved HLH
one responded well to steroid, other one started chemo
【在 I****a 的大作中提到】
: 33 y/o white male with history of type I DM on insulin, PVD, s/p fem/pop
: bypass 2 years ago who presented to local ER with bloody diarrhea, nausea,
: vomiting 2 days after he visited a casino and was reported eating a raw beef
: hamburger. He quickly developed AMS and was intubated for airway protection.
: His admitting labs were noted having a marked normocytic anemia and his
: blood smear was noted having significant agglutination. He has 3+ warm
: antibody presence on DAT. His hgb was in low 4s and he was transfused
: urgently with uncross matched blood. He also developed renal failure since
: day 2 of admission.
: ROS: all (-) except I mentioned above.
|
I****a
发帖数: 407 | 39 Ferritin was obtained initially however it was not quite high as what was
later on. |
