Shonkus, I wanted to revist this case we discussed some time ago, as I learnt something that could throw further light on the diagnosis. To begin with, here's the discussion so far. You are in bold and I am in italics.
Tnx....!!.Hope Ur in Gr8 spirits...FJS reported to me c/o Cough 2 mths, & occasional SOB with Fatigue.She is a housewife nd apparently looks healthy. Took t/t in 2010 for GERD & All.Rhinitis provided by then Physicain Dr Jagdish MD(Int.Med).Reports 2010 depict a normal AEC, ESR as 34mm/1st hr. Hb% was not done at tht time. on 18th instant upon detecting Pallor Inv.were ordered. Hb% was 7.5gm/dl, ESR 50 mm/1st hr, AEC 250, TLC 14,300/cmm, N-73%,RBC count 4.59mill./cmm, RBC indices :MCV 58.6 um3 (82-93),MCH 16.3pg (28-32), MCHC 27.9 g/l(32-36), RBS 95mg/dl, Throat C/s indicate Normal Flora. CXR PA Normal. She was surprised nd her husband attending to her complained of her irregular dietary habits. Upon , gentle prodding she gave a further h/o Bleeding PR since last yr on & off few episodes which she attributed to Piles,one episode 15 days back which stood controlled. Asked for, Thallasemia Profile, a stool Examination, Endoscopy, including proctosigmoido & Colonoscopy at a higher center. Ur discussion on this is solicited....Tnx....
SRL report is in hand FJS f/37 : Hb% 7.7g/dl , Hematocrit 27.4 , RBC 4.45mil/muL, MCV 61.6 fl, MCH 17.2pg, MCHC 27.9g/dl , Red Cell distribution width 21.4% , Platelet count 429thou/muL , Mean platelet vol 9.2 fl ,WBC count 13thou/muL , N 69% , RBC- Marked anisocytosis, mild poikilicytosis, microcytic hypochromic, with elliptocytes & ovalocytes. Serum Iron 97mic.gm/dl , TIBC 435 micgm/dl , % saturation 22 . Hb Variant Analysis : HbA 95.6% , Hb A2 2% , Hb F 0.3% , HbS,D,C 0%. Unknown Unidentified peak 2.1 (0.00 - 2.00 )....yes platelets here are high with raised total count....
Whenever I deal with RBC Indices the tall fair complexioned bespectaled ,well dressed Dr SB Pandey Asso.Prof Patho. who used to churn out these with monotonous regularity with a pronunciation as bland as it could be......nd believe me it seems as it is just yestday only I left the class...!!!....Golam case will post eve.....
The lady with the anaemia doesn't look as straightforward as it looks. While the tests would on the face of it, suggest iron deficiency, notice that while the TIBC is raised, the serum iron is in the normal range and that transferrin saturation is only slightly low, discordant with the severity of anaemia. Yet, it is almost certain that she has a degree of iron deficiency, as such marked anisocytosis & high RDW is not seen in pure thal trait.
However, notice the extremely low MCV and the very low haematocrit, and the relatively normal RBC count despite the severe anaemia. These are all features of thal minor. Yet, HbA2 is not raised. How do you reconcile this?
I believe this lady has a combination of iron deficiency anaemia and delta-beta thalassemia trait. Since delta chains are a component of HbA2, the latter has failed to rise. Thal traits are quite common among Asians.
I'd investigate the iron deficiency anaemia exactly as you have, but be aware that the response to iron therapy may not be as impressive as you'd otherwise expect.
To start from where we left off, I am convinced because of the reasons described above that this lady has a combination of thal trait and iron deficiency anaemia. But this time, I'd like to provide a bit more information, and perhaps a rider.
Firstly, I have learnt that iron deficiency anaemia (IDA) can calse false normalisation of the HbA2 in subjects with thal trait. When you treat the IDA, the HbA2 may rise above the upper limit of normal in these subjects. Although this lady's HbA2 is far below the threshold of around 3.2% considered diagnostic of beta thal trait, I'd repeat her Hb and HbA2 after a three week course of iron, say FeSO4 200 mg tds. The Hb should rise by at least 2 g/l in pure IDA, which I expect not to happen in this case because of concurrent thal trait. However, I'd expect the HbA2 to rise considerably- above 3.2%, confirming coincident beta thal trait besides her IDA.
There is however, one other possibility. The HbA2 would not be expected to rise in alpa-thal traits because the alpha globin chain is a component of HbA2. Alpha thal is coded by 4 genes, i.e. 2 paired genes on chromosome 16. If one or two genes are deleted, the patient hardly has any anaemia, but may have slight microcytosis. However, if 3 genes are deleted, the patient can have severe microcytic anaemia resembling beta thal.
Here's where her ethnicity comes in. Although alpha thal traits are well documented in Asians, particularly the alpha 1 trait or cis-deletion (--/alpha, alpha), beta thal trait is far more common in this population. In fact 8% of Bangladeshis carry the beta thal trait (4% among Pakistanis and 3.5% among indians, compared with only 0.1% in white British). On balance therefore, I feel this lady has a combination of IDA and either beta thal trait or beta-delta thal trait. You should get the answer if you repeat her HbA2 levels after a month on iron replacement therapy.
....needless to say,its a superb piece of analysis, which I am goin to pass it on to pt's.husband who is with her at KIMS,Trivandrum.This has again thrilled me further, to read Thallassemia nd other blood disorder,which I start tomorrow @ online Harrison which is my all time favorite book.Currently trending @ my clinic last couple of days: Pain Abdomen cases with history of 3-4days of fever,reporting low platelet counts, as low as 39,000/cmm as with Senaratne 45/m of SL. Incidentally all these DF pts presented with pain abdomen with fullness nd Masod,m/37 today complained of bleeding p/r upon defecation. Last few DF admissions r having pain abd.as p/c. Further, a couple of days back having wound up for the day @11.15pm,reception sought permission for an interview with an attendant who had brought a Fever case from Shaviyani Atoll, one of the atolls of the North, close to India. Asanul, m/37 had fever 15 days with extreme fatigue, chills, vomiting tendency,& only on
ReplyDeleteParacetamol, in a remote Island. The fever would come & go, making him v weak as he said. He presented with Fever nd Chills, with exhaustion, O/E: BP 90/60,P 92,RR22,T-38'C. Tongue Coated. System appeared normal. Though inclined to decline(as we had wound up) nd refer to IGMH,I decided upon his req. to admit nd thn refer to the higher center. Inv.were ordered.Bedside GRBS stood at 70mg/dl.He was put on IV DNS with Polybion,Inj.Avil,Efcorlin ,Cipro & Pantoprazole & Ondansentron all IV.except Inj .Avil which was given IM. At discharge time with the IV DNS pint, 3/4th thru a referral to the Nodal Hosp.IGMH( Indira Gandhi Memorial Hospital )for the nite ,he was nearly afebrile,the BP stood at 100/70,but to my shock, he woudn't respond to commands, with him alert nd awake, though, unable to understand routine verbal queries ,which hitherto an hr back he was able to while narrating his history nd having entered the clinic unaided. It appeared as if he didn't recognize me.The Deep reflexes nd Plantars were intact,there was no Neck rigidity nd Kernig's negative.The referral was ready nd he was sent to IGMH.I simply,lost track of the pt.due to poor co ordination nd pt.overload, nd learnt, today, thru a sister, whose husband wrks there, tht his condition is more or less the same,though stable but unable to comprehend whts being told to him...& is still under Evaluation...Ur suggestions as to wht happened to him tht nite....!!!...Tnx...
Thanks for these challenging cases, Shonkus. They really keep me going. I was reading something rather boring and struggling to hold my interest when your case zapped me back to life.
ReplyDeleteAsanul has relative bradycardia. With that temparature and particularly with that low BP, you'd expect the pulse rate to be a lot faster than 92. It is likely therefore that he has typhoid, which would respond to the ciprofloxacin you administered.
His rapid loss of ability to communicate is quite clearly due to one of the components of the infusate. While, one can only speculate so much, and it is impossible to be certain of the exact aetiology, I strongly suspect he developed muscular paralysis, probably periodic, because of hypokalaemia.
At least two agents that were administered were capable of causing acute hypokalaemia. His serum K would have been low to start with because of activation of the renin-angiotensin-aldosterone axis due to hypovolemia, vomiting and dehydration. The IV dextrose caused a surge of insulin release, which pushed the extracellular K further into the cells. IV steroids can also cause hypokalaemia albeit over a longer period, but more importantly, has been associated with the induction of periodic paralysis associated with hypokalaemia in subjects with thyrotoxicosis.
Thyrotoxic periodic paralysis, which is essentially due to hypokalaemia, occurs almost uniquely amongst Asian males of this age. I suspect this man had undiagnosed thyrotoxicosis and developed hypokalaemic periodic paralysis because of the above factors. At its most extreme, such paralysis can last for days. The person can hear you, but cannot respond because of flaccid paralysis. The hypokalaemia and associated Mg deficiency needs to be corrected expeditiously, and if thyrotoxic, and once BP has been corrected, IV propranolol can help, because the mechanism involves a catecholaminergic shift of K from the extracellular to the intracellular compartment.
As a rider, I'd add that at 70 mg/dl, the patient wasn't hypoglycaemic, and as such 0.9% saline or Ringer's lactate would have been an ideal initial infusate, with K added based on serum or VBG potassium.
Please let me know of his progress if you can. Thanks for the stimulating case.
....Tnx nd superb again...yes, my initial reaction was as to the infusate...which was handily available as Pharmacy had wound up at tht hr & frankly I didn't suspect Hypokalemia...normally in normal hrs ...the lab will immediately give the results, in his case it was late nd Inv.though ordered were not done as quick referal was made...today will follow it up with usual channels nd let u know....Tnx a ton once again...!!!...happy tht these cases r of some use to u....!!!
ReplyDelete....Abul kashem 50/m frm Gasfinolhu Island Resort,presented with hiccoughs an yr back which wouldn't go...he was in the habit of taking pain killers OTC for a L sided,throbbing lancinating headache of long standing....he had fullness of abdomen with vague pain,constipated nd this hiccoughs which won't go...Inj.Perinorm with Phenergan somewhat controlled it but again on revisit the nxt day the hiccoughs were present....Ryles tube Intubation & aspiration with a 50cc syringe almost immediately controlled the Hiccoughs....the aspirate was muddy rust color....Inj Pantaprazole worked wonders and he was relieved though the headache persisted which was more localized over L temporal area...Xray PNS was normal...after a couple of weeks the headache was dull with burning shooting sensation across the face...CT Brain he got done as per his req. nd was normal....his blood parameters were normal...put him on Tegretol 200mg BD along with Amitryptiline 10mg for a mth which reduced the intensity nd he continued, taking the same medicine as such for nearly a yr, happy nd satisfied, only to report with increased intensity of previous sensation for the last one mth over L facial area....considering increasing dosage of Tegretol...& confused about Gabapentin 300mg & possibly Phenytoin....being treating him for Trigeminal Neuralgia....Ur thoughts & latest info. nd actionables on this ...Tnx...
ReplyDelete...A very Gud morning...!!!...wanted to wait for some time but if u so remember the Hyperthyroidism case of Abul Kalam Khan m/24 wherein I had prescribed Neo mercazole nd subsequently settled for a line on De Quervain's Thyroiditis...Neomercazole stood cancelled the v nxt day.Today after a span of 27 days he has reported back with vague pain Medial border of Rt.SCMastoid with vague swelling nd h/o fever on & off.Current Exam _ P 110, BP 100/70, T- Normal. The previous Inv suggested +++ B strepto in throat c/s to which he took Cipro , & the Thyroid profile was T3 270.17ng/dl,T4 19.10mu g/dl, TSH 0.01mu IU/l. Prov .Dx De Quervain's Thyroiditis,Hyperthyroidism , URTI . He doesn't show any sign of Exopthalmos, Tremors . Have asked for a Rpt Thyroid Profile for tomorrow....Will De Quervaine's Thyroiditis persist after nearly a mth?..also last time I had put him on Prednisone ...Ur val.Inputs...Tnx..
ReplyDeleteShonkus, wrt Abul Kalam....is he still on steroids or has this been weaned off? Clinically, he still seems to have ongoing thyroid inflammation and appears thyrotoxic. I suspect his steroids may have been weaned too fast. Please check his ESR/CRP as well his TFTs, and add or increase steroids, as may be applicable. I'd recommend at least 20 mg prednisolone at this stage, reducing by 5 mg every two weeks.
ReplyDeleteRegarding the first patient, I completely agree with your diagnosis of trigeminal neuralgia. I'd stick to tegretol and increase the dose in 2 steps to 400 mg BD. Would not try either phenytoin or gabapentin at this stage. Please monitor blood count while increasing carbamazepine.
Thanks for the cases!
...Tnx...yes Abul kalam was on Wysolone @ 20mg for the 1st wk, 10mg for the 2nd, 5mg for the 3rd...I think it was underdosage...or may be he didn't comply....as monitoring was difficult as he belonged to HuluMale a satellite Island township....yes will order ESR & CRP tomorrow as tomorrow is the earmarked day for SRL sample of TFT ....on Wed & Sun we send thru DHL to Mumbai...I hv totally forgotten as to wht happens to the count whn u increase dose of Carbamazepine....Epilepsy I had dealt more frequently @ Island Health Centre, whn I had an initial stint there....tht particular Island of Fonadhoo & the concerned Atoll of Laam one of the southernmost, near to the Equator had a catchment area of pts.frm Dambidhoo, Isdhoo, Mavah, Mabaiidooh, Guraidhoo, Gadhoo Islands in addition to Fonadhoo the capital Island whr I was posted 8yrs back....the regional Hosp.was at GAN...u may Google Earth nd chk these remote locations.....these Islands had sizeable Epilepsy,Schizophrenic pts. nd Psychiatry was routine practice thr...with depression ,anxiety abundant as a result of the aftermath to the Tsunami whch hit thr.....the hitherto peaceful people had no brush with major disaster or calamities,also very rare incidence of abnormal deaths like Murder or RTA as compared to Indian Mainland, hence I felt thr Mental defenses were poor nd they were ill equipped to face anything difft frm routine....resulting in lot of Psychiatry cases.....in contrast Male' is difft. with its populace smarter...just a couple of hrs ago Md.Shahjahan m/29, who had complained of fatiguability, loss of wt, & freq.of micturition shocked me with this yrs all time clinic high FBS & PPBS which stood at 399mg/dl nd 800mg/dl with Ketone bodies ++ nd, Sugar ++++ in urine...I hv formulated an immediate mgmt. would like to hear ur expert opinion nd advise & whether it is prudent to treat him on OPD basis as we shut down aft.11pm every nite,with all pts discharged nd recalled nd reviewed nxt.morn....my charge of 10 beds r filled up thru out the day with short admissions, treatment, nd compulsory discharge for the nite with referral to IGMH if any case warrants such....or else following day the cases revert back @ 8.30am....with this blood sugar level, how best he can be managed here given tht he is unwilling to go to IGMH...ur thoughts for this remote area, logistically, difficult practice....!!!!...Tnx....
ReplyDeleteShonkus, thanks for the well written perspective. You certainly have a grasp of the local demographics, which is pretty important for a physician.
ReplyDeleteAbout Shahjahan, he's too ill to be managed on an outpatient basis. He has DKA, and with that level of blood glucose, is badly dehydrated. Bad things could happen.
He needs assiduous management. Blood gas, followed by IV insulin through a pump, lots of IV fluids, with potassium and possibly IV antibiotics if there's an infection, round the clock monitoring for a day or two. Best cared for in a more acute facility with nursing resources.
Please let me know of his propgress.
....since our Hospital is a 15 hr hospital, Shahjahan was transferred after initial t/t to IGMH, whr he is currently nd expressed his desire to revert back as soon as Doctor's there release him....I reassured him nd told his attendant to be in constant touch....recent experience with DF admissions, pts r afebrile, stable, ambulatory ,but even after a week with Pulse pressure wide,Proper hydration & Urine output ,the Platelet count continues to get lowered, sometimes abominably low I thought in few cases nd moderately low in most...few reported intense Pruritus with redness of skin generalised & Shapon reported Epistaxis...I am interested in the mechanism of lowering of Platelets nd how its increase is not in direct proportion ,rather inversely proportional, in some cases, as it appeared, I thought, to the symptomatic relief nd constitutional betterment & seems not linked to the recovery phase of the fever. This creates heart burn nd anxiety to the pts. who r fairly recovered but only visit to track their Platelet counts nd feel dissapointed whn they see it going further down, even if they feel much much better nd without Fever....Ur analysis....Tnx...
ReplyDeleteI believe platelets are innocent bystanders in DF, destroyed by cross reacting antibodies which the body ineffectively makes against the Dengue virus. Once the antibodies are in place, and there is complement fixation, destruction of platelets will continue apace even as the virus recedes.
ReplyDeleteI believe the real killer in DF is not thrombocytopenia, but plasma leak syndrome and coagulopathy. There is overemphasis on monitoring low platelets, when the stress should be on correcting haemodynamic compromise and coagulopathy. Platelets really need no correction until the count has fallen <20,000, which is pretty uncommon.
.....well analysed...agree in toto....its a fever v v interesting....!!...Tnx..!
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