DOUBLE WHAMMY DR K.JAGADEESWAR REDDY DNB MEDICINE

Transcription

1 DOUBLE WHAMMY DR K.JAGADEESWAR REDDY DNB MEDICINE

2 52 yrs male who is hailing from Mannargudi of Tamilnadu Chief c/o excessive fatigue, exertional dyspnea, black coloured stools on and off, bleeding from the gums during brushing, dark yellowish discolouration of urine,generalised weakness since august 2018

3 HOPI H/o black coloured stools on and off -2-3 episodes per week since aug 2018 and not associated with vomiting of blood H/o dark yellowish discolouration of urine since aug 2018 initially then followed by yellowish discoloration of eyes since 1 month,progressive in nature,not associated with fever,abdominal pain and distension

4 H/o bleeding of gums during brushing since 2018,not associated with skin rash,petechiae,purpura and generalized itching H/o breathlessness on exertion since aug 2018,progresssive in nature,not associated with chest pain,cough, palpitations, sweating, PND, orthopnea,lower limb swelling

5 H/o easy fatiguability present since aug 2018,progressive in nature,initially able to do household activities and office works and later as days progressed unable to do similar activities as previously for which he visited the hospital H/o of loss of appetite since 2 months but no h/o weight loss

6 Past History Hb-6gm/dl,PLT -8000cells/ccmm Aug 2018 cause was not known!! OGD done 1½ Month ago- Diffuse errosive gastritis / esophagitis - H.pylori which is negative by RUT kit, advised PPIs, antibiotics, multivitamins - Has not taken the treatment

7 No H/o prior surgery and hospitalisation No H/o any prior blood transfusion in his life No H/o DM,HTN

8 Family H/o No h/o similar illness in the family Personal H/o consumes 6-8 quarters of brandy per month since 20 yrs Non smoker Diet H/o mixed diet No H/o any Drug intake

9 Examination conscious,coherent,well built and nourished,bmi -24.1kg/m2 Vitals :BP -100/60 mm Hg Afebrile PR -110/min, regular RR -16/min, SPO2-86% in RA, 96% with 3 litres O2 Pallor +,icterus present No cyanosis,clubbing, lymphadenopathy,pedal edema

10 Abdominal examination All quadrants moving equally with respiration Soft, No hepatomegaly,mild splenomegaly present-2cms below costal margin 5 th ICS dull note liver border BS present,no hepatic or arterial bruit P/R examination no blood stained feces CVS S1S2 present,no S3 or murmur RS and CNS NAD clinically

11 Alcoholic,Pallor,jaundice,mild splenomegaly are significant findings Provisional diagnosis : Anemia probably due to GI bleed? Hemolytic anemia

12 As he walked into the casualty in a stable state, he got admitted into ward initially and workup for anemia started

13 As his Hb -3.4gm/dl severe anemic and he is requiring O2 support and chances of heart failure due to anemia are more,he moved into ICU for further management

14 Investigations On Admission CBC Hb -3.4gm/dl,TC 4,200 /cumm -PLT 20,000cells/cumm MCV -133 fl, ESR -45 mm/hour PS blood smear Pancytopenia with dimorphic anemia, Polychromasia with anisopoikilocytosis LFT T.B -4.5,In.B-3.8,DB -0.7,SGOT 106 /SGPT -97/ALP-59,INR-1.50/PT 16.5 sec,albumin-3.8,globulin 3 RFT Cr -0.57/Na /K+ -3.7/Chl -103/BUN -15 RBS 258 mg/dl TSH HIV,HBV,HCV Others 2.2 miu/l -negative BT -4 min 15 sec /CT -9 min 30 sec

15 Reticulocyte count -5.7% Reticulocyte production index 0.6 LDH U/L Haptoglobin -<10 [30-200mg/dl] Coombs test negative PNH and G6PD test reports are awaited

16 Vit B12,folate and iron sent and reports awaited Treated with O2 inhalation, PPIs, IV vit K,IV tranexamic acid CXR - B/L mild pleural effusion USG Abdomen - Mild hepatomegaly- Normal echotexture pattern and mild splenomegaly -13 cms. ECHO Mild con LVH with good EF -60% and mild TR Meanwhile packed cell and platelet transfusion was done

17 Stool occult blood is negative Gastroenterolgist opinion sought who suggested no requirement of endoscopy at this moment with low HB and thrombocytopenia as previous OGD done 2 months prior

18 Investigations- day 1 Vit B12 120pg/p g/ml [ ] folate 1.22ng/ml[ ] serum Iron 367 mcg/dl[ ] UNEXPECTED!!!! iron profile awaited MP smear, Dengue serology negative Hb-4.7gm/dl,Plt -30,000

19 BM biopsy was planned in view of pancytopenia due to BM failure,but postponed for later date b/c of low PLT and deranged INR Vit B 12 supplements and folate supplements were started

20 Hb-6gm/dl,PLT-60,000,TC Day 2- Iron studies Sr iron -367mcg/dl TIBC -279 mcg/dl[ ] Ferritin -833ng/ml [ ] Transferrin saturation -131% [25-50%] iron overload picture

21 Day 3 Hb -8.4/PLT-20,000/TC -2000cells/cumm Inspite of deranged INR and low PLT,BM biopsy done under aseptic conditions under Platelet coverage as there pancytopenia d/t BM failure and deranged iron profile HB electrophoresis study normal

22 Hb electrophoresis Normal

23 Day 4 Hb -8.8,PLT -30,000/TC -2,290 cells He was transfused totally 4 units of packed RBC and 4 units of SDP

24 BM BIOPSY SLIDE Ringed sideroblasts

25 Day 5 -BM biopsy report Pancytopenia with cellular bone marrow with nucleated RBCs of 74% and increased iron stores with ringed sideroblasts -25% with erythroid hyperplasia - M:E ratio of 1:3.08[ 3:1]

26 Sideroblastic anemia

27 PNH flowcytometry

28 G6PD test

29 Pyridoxine supplements were added Possibility of MDS was considered - Testing for MDS was not done at this moment as patient is improving with vit B12 and folate supplements.

30 Advice on discharge Hb -9.7gm/dl,PLT-35,000,TC -3,700 vit B12, folic acid and pyridoxine supplements

31 Follow up after 2weeks Hb-10.8 gm/dl, TC -4,800/mcl, PLT -3.3 Lac Advised to continue B12,folate supplements

32 Diagnosis 1) Hemolytic anemia Vit B12 deficiency 2) Pancytopenia with cellular BM - Vit B12 and folate deficiency - Alcoholism 3)Sideroblastic anemia Alcoholism and folic acid deficiency Myelodysplastic syndrome to be considered

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