Hemoglobin 4.2 mg/dL due to Autoimmune Hemolytic Anemia
Author: A. Rajaminackam, M.D., Department of Hospital Medicine, Cleveland Clinic
Reviewer: V. Dimov, M.D.
A 51-year-old female has had fatigue, weakness, and shortness of breath (SOB) with exertion during the past 4-5 days. She called her primary care physician (PCP) who recommended that she had her hemoglobin checked. He called her back with the results, and told her to go to the emergency room (ER) for further treatment of severe anemia. On admission, the patient denied abdominal pain, chest pain, congestion, nausea/vomiting/diarrhea/constipation (N/V/D/C), dysuria, headache, chills, hemoptysis, neck pain, rash, or sore throat.
Her symptoms were exacerbated by activity and relieved by rest and laying supine. She also felt palpitations intermittently.
Past Medical History (PMH)
Diabetes type 2 (DM2).
Medications
Lantus (Insulin glargine, rDNA origin) 25 mg SQ QHS, Humalog (insulin lispro) SSI SQ with Accu-Chek Blood Glucose Monitoring TID.
Physical Examination
VS: mild tachycardia, no hypotension.
General appearance: pale, non-icteric.
Eyes: EOMI, PERRLA, sclerae non-icteric.
ENT: Oropharynx clear, no plaques or exudates.
Chest: CTA (B).
CVS: Clear S1S2.
Abd: Soft, NT, ND, +BS.
Ext.: no cyanosis/clubbing/edema (c/c/e).
Neurologic: AAA x 3.
No lymphadenopathy.
What is the most likely diagnosis?
Severe anemia that is symptomatic with fatigue and shortness of breath (SOB).
What are the most likely causes of anemia in this patient?
Blood loss?
Hemolytic anemia?
Iron-deficiency?
What laboratory workup would you order?
CBC+Diff
CMP
Reticulocyte count
LDH
Haptoglobin
Peripheral smear
What happened?
The hemoglobin (Hgb) was 4.2 mg/dL, MCV 144 fl, and reticulocyte count 41%. The patient most likely has hemolytic anemia.
What other tests would you order?
Direct and indirect Coombs' test
ANA, RF
C3, C4
Chest X-ray (CXR)
CT of the chest, abdomen and pelvis (CT c/a/p)
CBC and CMP (click to enlarge the images).
Immunology tests (click to enlarge the images)
What happened next?
The patient was admitted to a regular medical floor and a hematology consult was called. The direct Coombs' test was reported as positive.
The CXR and CT scans were negative for neoplastic disease.
The patients has autoimmune hemolytic anemia (AIHA) mediated by warm antibodies because the hemolysis is observed at normal body temperature. By contrast, in the cold antibody AIHA, the autoantibodies attack the red blood cells only at temperatures significantly below normal body temperature, e.g. when working outside in the winter.
Would you transfuse this patient?
The hemoglobin was 4.2 mg/dL and if the patient was symptomatic. A blood transfusion was indicated.
In general, it may be difficult to find compatible blood in AIHA because of the presence of autoantibodies. RBC transfusions are generally avoided unless absolutely necessary.
How would you treat this patient?
Solu-Medrol (methylprednisolone) 100 mg IV q 6 hr.
Consider immune globulin infusion.
Follow-up on the Hem/Onc recommendations.
Hemoglobin response to steroid treatment in AIHA. Taper glucocorticoids very gradually to avoid a relapse of hemolysis (click to enlarge the images).
Final diagnosis
Warm Antibody Autoimmune Hemolytic Anemia (AIHA).
References
Coombs' test (click to enlarge the diagram). Source: A. Rad. GNU Free Documentation License. Wikipedia.
Hemolytic Anemia. eMedicine.
Autoimmune Hemolytic Anemia. Merck Manual.
Seasonal Hemolysis Due to Cold-Agglutinin Syndrome. Lyckholm L. J., Edmond M. B.
N Engl J Med 1996; 334:437, Feb 15, 1996. Images in Clinical Medicine.
Further Reading
Robin Coombs, 85, Inventor of a Diagnostic Blood Test, Dies. The New York Times, March 27, 2006
Published: 05/20/2007
Updated: 03/18/2010
Labels: Hematology, Oncology
48 Comments:
I was also diagnosed with warm auto-immune anemia and after three months of illness have finally reached a hemoglobin level of 11.2. Prednisone did not help nor did removing my spleen.Rituximab saved my life.I also had a hemoglobin level of 4.2.I'm glad its all over with.
Jeff Sergeant/LeClaire,IA
My sister also was diagnosed with AIHA and a hgb of 5.0. Four transfusions later, along with prednisone 70 mg and Rituxan, her hgb is normal.
Does AIHA tend to come back/flare up with recurrent episodes?
Also diagnosed with Idiopathic Auto-Immune Hemolytic Anemia. Went to the ER with Hg of 63 (normal range 140-180). No luck with 3 months of Prednisone (except future side effects I'm sure) and splenectomy didn't "fix" the problem. IG IV treatments help, my Hg has stabilized, but not fully cured. Western medicine... anyway, trying some acupuncture and other ways to remedy the problem.
My father has aiha and his hgb level was terribly low also.He is currntly on Prednisone and just had his 6th rbc transfusion.Dr. wants to remove his spleen next.I will ask the Dr.about the human (cd20) clusters of differentiation molecules and see if my pops can skip that splenectomy stuff altogether.Anyway,I appreciate this site and your peoples feed back!I hope it works out well for him.
Diagnosed with Autoimmune haemolytic anemia 6 months ago. Placed on prednisone.After hematologist tried decreasing the dosage the blood started hemolising. Hb had dropped to 3.5 now it is 9.0 after transfusion.
No particular reason given for Anemia. interested in knowing whether joint pains and swelling can involve anemia?
I am considering marriage with a girl having AIHA, will we have a normal life, children etc.
Hi,
Does anyone out there have Idiopthathic Autoimmune Haemolytic Anaemia with a Negative Coombs?
I have been on Prednisolne for 2 months when blood dropped to 4.
Now they are weening me off the Hb is starting to drop again. Rituximab is the next step, anyone been on it?
Hi,
me too, had prednisolone for about 4 months, multiple transfusions, immunoglobulins blood count of 4...last resort rituximab, fantastic stuff, saved my life and sanity
I am writing this from a chemo clinic, in which I am now receiving my 4th Rituxan treatment. After approx. one month, 4 transfusions, treatments, and a recent discovery of a blood antibody I am starting to worry. Hematologist has recommended Splenectomy after HGB levels continue to drop average of one point per 4 days -- currently at 6.8. Anyone receive cytotoxin before the splenectomy?
1 month ago Dx with Aiha hgb 4, im 28 did not respond to prednisone, or Iga then went to Rituximab X4, with little resulsts by 3rd dose hgb down to 5, back to hospital they took my spleen- laperscopically(3 1in incisions) quick recovery, 2 weeks later incisions basically healed and getting around like normal, hgb now 13-almost normal! hopefully I won't drop again dont want to go on to the bigger badder chemo drugs
my sister was diagnosed with AIHA and shes given prednisone.. hope she'll be okey..
Diagnosed back in March 07 with AIHA. 5 blood transfusions and 6 months of predinisone, still sick.
Started Rituximab last week, have 3 more treatments and hoping it will work. Can't remember the last time I felt well. Have had all the tests to rule out any underlying diseases. Bone marrow biopsy the worst test. Lowest hemoglobin 5.6, highest 11.3 over past 6 months.
a patient suffering from AIHA is being treated with mild doses of dexamethasone 8mg a day there is an improvement of Hb from 5.6 to 7.0 but she has the problems of gastritis and constant increase of liver enzymes can u say what could be best for her
I have warm AIHA and have twice had Rituximab - last time because Hgb dropped to 5, I had pleural effusions and circulating sepsis - rsulting in peri-arrest. Rituximab prescribed whilst I was still on 60 mg of prednisolone daily. My Hgb level now 14.3 (!!!personal best) and pred. stopped. Rituximab works well for me and I have no side effects which is lucky as I have 2 other auto-immune problems!
I have this too. Have been at 3.5 hg twice. I too cant remember the last time I felt well. Currently have lost a lot of my life and support systems because people around me can't live with this illness going forward. Any daily routine measures that can make me feel better?
My brother was diagnosed with Autoimmune hemolytic anemia as a small child, 3 years old. They tried steriods. They did not work. They then removed his spleen and everything was fine. He went into surgery white as a sheet and came out with color. I thought that children only got this and it was very rare. I'm very surprised to see that adults are getting this and spleen removal is not working. I'm wondering if my brother's will come back? He still has his blood checked once a year and so far he is fine.
And they did think when he was young that his liver could start doing what his spleen did???
I am sick and tired of being sick and tired . . . diagnosed spring 07 with cold hemaglutinin syndrome-- hemoglobin ranges from high 90s down to 50s. Started with plasmapheresis and transfusions, and found that IVIG helped to keep the wolf at bay for 6-8 weeks at a time. When I get sick, I really get sick, and require the whole kit and kaboodle to bring me back. Next, is the rituximab--should I write my will? JR--Hamilton, Ont.
My daughter had AIHA at 2 months of age. After about 20 transfusions in 3 months, prednisone (which didn't do anything) and finally cellcept (which is also used for organ tranplant patients-stops the immune system from rejecting the organ, basically shuts it down) she recovered. No official cause for the illness or the recovery (she's 4 years old now). I believe, and her hemotologist finally admitted as a possibility, that it was due to her body's response to getting a pertussis (whopping cough) vaccination while she had a slight respiratory illness (a cold). My other daughter (6 mo.) has been fine, but has not and will not get the pertussis vaccination. The drs. say I have no reason to believe the AIHA will ever come back.
Diagnosed Feb 07, was down to 5.2, initial treatment was blood transfusions received 16 in total (I live in Spain) developed pneumonia within hours of being in observation, was diagnosed possible mycoplasma. Received antibiotics and 120mg of prednisone. Levels didn't go further than 9.0 hg, so then wen ton to receive 19 sessions of plasmapheresys... am now on 150 mg of azatioprine and 5mg of folic acid, came off pred, but have had 2 set backs since then. Have been good for 2 months now without blood tests, it's the longest I've been feeling well.
Drs told me my AIHA will always stay with me and we are only keeping it at bay, trying to save my spleen. I'm only 29, but if I didn't take my aztioprine I would die. I have written my will, planned my funeral. the most menacing me for me is any infection in the lungs as they are very weak. This illness has taught me every day is a gift. I am very grateful to my hematologists 4 of them, they have spent months trying to control it as I don't follow any logical patterns neither am I cold or warm or have had anything that could have caused this, I know they spent and amazing amount of money on me and yet they treat me like my case is personal to them. View my blog, click on my name!!!
I have AIHA (warm) and am doing well after 6 months of treatments. I have had my gall bladder removed, and got worse. My hgb dropped to 4.6. I have recieved gammo globulin, vincristine and retuxen. Also, 5 units of blood and lots of cancer scares. I was on 80 mg. of steroids per day for 4 months. I'm off of them as of yesterday. I hope I remain healthy. I am sick and tied of being sick and tired. People look at me and can't see anything wrong and wonder if it's all in my head. My doctors are great and I hope to remain healthy. INDIANA
Hi,
I was diagnosed with warm AIHA last december with Hgb at 4.0. I was wondering if any of you had their teeth whitened or bleached? I believe there is a correlation here, the ingredient in these products are either carbamide peroxide or hydrogen peroxide. I relapsed twice and I remember bleaching my teeth around that time and also once shortly b4 first diagnosis. I'm having my spleen removed tomorrow, and hopefully that takes care of the problem.
I've had IAHA since I was a child, now 30. Steroids are no good, rituximub didn't work. Immunuglobulin with cyclosporin seems to work but after 6 months IAHA was back again and the side effects to the immunuglobulin have been horrendous, they are now suggesting removing my spleen, any one got any info on success rates if I were to proceed with this course of action (my options are now very limited)
Its strange but the comment made about teeth whitening is very familiar. I got ill soon after using a teeth whitening pen..... it may just be a fluke. I dont think it has anything to do with it. I went on holiday to USA and got bitten all over my feet. I am wondering if this had anything to do with it?.
I have been ill for 4 years now and I am 26 years old. I am on Azathiroprine 100mg per day. I used to be on steroids and that made me feel awful. My blood went down to 3.5 and I was made to walk to a hospital in London! by doctors. I have had several operations - gall bladder removed - endoscopy and sphincterontomy. I am basically being kept alive by my drugs at the moment. Drs just dont know what is causing it or really how to cure it. There is no diagnosis. My Coombs test was negative as was bone marrow. After having two bone marrow biopsies,still remained negative.
Has anyone else got any idea what this may be or any cure? or have you got this?.
I am desperate to get back to a normal way of life.
My son became very ill with severe onset of AIHA [3.8 hg] and enlarged liver/spleen; indirect bilirubin was 4.1. He was perfectly healthy and breastfed. this came 3 days after his vaccinations and I believe it was due to the vaccines. After 8 mo. of pred and IVIG therapy and one transfusion, he was Coombs negative and never suffered another relapse. Anyone out there with a similar story?
Hi, i was recently diagnosed with autoimmune hemolytic anemia. I'm 23. I was down to heamoglobin level of 26 when i was admitted to hospital last 3 weeks. Normal count would be in the range of 115 - 155 g/L. I was in hospital for a week. Had 5 bags of blood transfusion and heaps of Octagams. Been out of hospital for nearly 3 weeks but have blood tests twice weekly as doctors are monitoring my blood level but blood count seem to drop everytime. My recent blood test was at the count of 80 and all of the anemia symptoms came back (severe headache-thumping in the head,nausea,fatigue,dark urine...) so yesterday I received Octagam drips (Immunoglobulin) of 150ml. I'm currently on Prednisolone (50mg) and Acid Folic supplements and just yesterday my doc has prescribe me with Azathioprine since the Prednisolone didn't seem to work. I didn't think that this blood disorder was that serious but after some research and reading it seem to be pretty serious. I'm also terribly worried bout the side effects of the drugs and medical treatments, internally, physically and emotionally. Can anyone out there comment on their experiences.
I also have autoimmune hemolytic anemia. I was admittied to the hospital 6 weeks after my son was born with a hemoglobin of 2 and continued to drop. I have tried prednisone, cellcept, and now trying Azathioprine. So far nothing has worked I havent met anyone with both cold and warm antibody autoimmune hemolytic anemia. I'm very concerned and just wondering if anyone else is in my shoes??? Along with all this I also have Lupus,high blood pressure,hypothyroidism,and Rheumatoid Arthritis everything but the thyroid issued occurred after the anemia started.
If anyone could point me in the right direction for my fathers sake I would greatly appreciate.
My father has been diagnosed with a severe cold anitbody. We live in one the hotest states there is, Louisiana. Since being diagnosed in August, my father has had over 40 transfusions, his count got down to 3.9 at which point he coded in the emergentcy room. His iron levels are so high, he is now on Exjade to reduce iron levels. His doctors say there is nothing that can treat the cold antibody.
I just want to know is there anyone that has any success with any kind of treatment for this disease. Please let me know.
i had autoimmune haemolytic anaemia after i had chemo and radio... rituximab saved my life, i had 3 days to live but the stuff saved me xxx
I need help too. My son is 14 and been on prednisone for four months...not working!
Please see your doctor. This is not a website for medical advice.
My email is thmarine1@yahoo.com if you have any advise for us. He has warm AIHA. Docs want to try cellcept and I'm so scared about the side effects. I can't find a support group or info on this terrible problem. I really need some help!
I had surgery on a perferated duodonal ulcer and my hemoglobin went down to 4.2. It went untreated and I suffered hearing loss from nerve damage to my left ear. When I saw my regular doctor after being released from the hospital he ordered a CBC. My hemoglobin count was 9. He put me on iron. My hemoglobin count is normal now, but I will probably never regain the hearing in my ear.
My 15 year old daughter was diagnosed with AiHA last year. She has been on prednisone for quite a while. We are switching to 6-MP because of some serious mood problems that might be added to because of the prednisone. I would love to connect with others that can share their experiences.
I was diagnosed with a mixture of both warm and cold antibody strains (how, I don't know) of AIHA in 2003, when I was 16. Was nearly comatose when I went to the ER. Had a hemoglobin of 32 (Australian measurements. . /extremely/ low). Was treated with blood transfusions and prednisone. I had a splenectomy (unsuccessful), as hemolysis would flare up again whenever we tried to wean down the prednisone. I was eventually treated with MabThera, and made a slow recovery. I still have very minor hemolysis, but it's slow enough that it doesn't affect me.
I am a 76 yr female. Diagnosed with Myelodysplastic Syndrome,Autoimmune Hemolytic Anemia, Severe Neuropathy and Rheumatoid Arthritis. Have been RBC transfusion dependent since 2003 (2 units/mo). RBC transfusions becoming difficult because of autoantibodies making it difficult to find compatible blood.
Hemotologist is recommending a splenectomy. I just don't feel that it will help and will still need the transfusions. If anyone has the same problem--transfusions hard to match and have Autoimmune Hemolytic Anemia. Did splenectomy help?
Previous post
I've had IAHA since I was a child, now 30. Steroids are no good, rituximub didn't work. Immunuglobulin with cyclosporin seems to work but after 6 months IAHA was back again and the side effects to the immunuglobulin have been horrendous, they are now suggesting removing my spleen, any one got any info on success rates if I were to proceed with this course of action (my options are now very limited)
Update
I had my spleen removed 8th March by key hole surgery, the operation went well, so far my HB has stayed at no less than 12 which is amazing. I will be completely off Cyclosporin hopefully next week, have my fingers well and truely crossed that this is the cure!
I am a male (48) who was taking simcor for about 6 months and tylenol when i felt a cold comming. I began seeing my urine dark and climbing stairs made me dizzie and felt tired. was diagnose AIHA (WARM) Had two blood transfusion now in my house taking prednisone 80 mg a day its been 4 wks.and when i got out of the hosp. was 7 Hemoglobin count each wk it goes up one point now i am at 8 doctor tells me after 6 wks they will lower the dose and see how the body reacts. hoping for the best! Ricky from Puerto rico.
Hi,
I'm a 51 yr old male, with Coombs (DAT) Negative AIHA since early 2005. Tried almost every treatment - Pred, Danazol, Cellcept, 12 Rituzan infusions -nothing helped. I dreaded this but finally had my spleen removed June 3. HGB went from 7 to 12 in 12 days... Feel so good, first time in four years. Running again, hitting the weights. Hopefully, will be this way for the rest of my life.
For anyone suffering from AIHA, I highly recommend splenectomy.
Ernesto
Male, 35, began having autoimmune events since 1998. After all these years of treatment mainly with prednisone and having had splenectomy in 2001, last October my hgb dropped to 4.2 and had to rush to the ER. IV prednisolone shots and dexamethasone began working three days after, but the final treatment was Rituximab (two infusions in one month). Dong well right now, hope the effect lasts another 10 years!
I'm a 36 year female who has been dealing with IAHA for 2 years now. I never had the chicken pox so in 2005 I had the varicella vaccine. A few days later, I broke out in a rash that never went away. I still have it on my arm and it only stays in one little area. I know the hemolytic anemia developed from this vaccine and knew at the time I should not have received it but did it anyway. I went up and down on the prednisone and finally found an great physician who knows how to treat me. In Feb I completed four rounds of Rituxan and have been on 7.5mg of prednisone. I feel good but always worry my HGB will drop again.
I'm just wondering if their is anybody who had hemolytic anemia and on low doses of prednisone who have become pregnant. My physician told me it should be fine but I am very afraid to become pregnant with a autoimmune disease? Thank you!
My mother has AIHA and she is 83. She has had apx. 5 transfusions, immune globulin therapy, and is now on 30 mg of prednisone per day. Her hemoglobin is now 7.3. After she goes off the pred. they want to give her Rituxan. Does anyone know of anyone that is elderly that has received this drug? Have any of you that have had this treatment gotten sick from it? I'm so afraid it will be too hard on her. Appreciate all comments.
i am 43 year old female, i have had AIHA since i was 17. i had a splenectomy when i was 19. all seemed quite good for a few years, then i had a relapse. since i have had a number of relapses. i have tried prednisolone, azaphiaprine,cyclophosphomide,immuno globulin,
i am now on my third 4 week dose of rituximab,as i relapsed over christmas,spent new year having a blood transfusion.
testing testing, wrote a big comment but didn't get posted
Had autoimmune hemalytic anemia 5 years ago. Hemoglobin was at 4.2. Was given 3 transfusions then on/off with prednisone. Did 4 treatments of Rituxan. After 3 years my hemoglobin was up to 12.0. Last October hemoglobin was back down to 4.0. On prednisone again. We'll see how I do on that. If it starts going back down again, may have to do the Rituxan treatments again. Can't figure out why this is happening.
AIHA is a fluctuating disease process with the activity of the condition going up and down as the time progresses. This explains some of the unpredictability in the variations of your hemoglobin level.
Previous post
I've had IAHA since I was a child, now 30. Steroids are no good, rituximub didn't work. Immunuglobulin with cyclosporin seems to work but after 6 months IAHA was back again and the side effects to the immunuglobulin have been horrendous, they are now suggesting removing my spleen, any one got any info on success rates if I were to proceed with this course of action (my options are now very limited)
Update 1
I had my spleen removed 8th March by key hole surgery, the operation went well, so far my HB has stayed at no less than 12 which is amazing. I will be completely off Cyclosporin hopefully next week, have my fingers well and truely crossed that this is the cure!
Update 2
Well it's a year since my spleen was taken from me and so far so good. I've been battling this disease since I was 8 years old and I am trying not to believe too hard it's actually gone away for good just in case - but a whole year no medication and healthy I just can't believe it!
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