PREFINAL EXAM PRACTICAL

40 year old women who  worked as farmer till last year ,she has to stop working because of her health issues .

She was married at the age of 15 years.Mother of 3 children .All pregnancy were uneventful .she had 2 LsCs surgeries.She recieved blood transfusion for the first time during her last child birth.

She was apparently assymptomatic 8 gets back and then she presented to our hospital with history of 1 month of fever ,15 days of jaundice and pedal edema.Bleeding per rectum and mass per rectum ,history of Malena since 1 week .

On investigations - she was found to be severely anemic with Hemoglobin - 2.6 ,low platelet count - 50000.She received 3 PRBC transfusions then her hemoglobin improved to 7.4 but her platelet count was 40000 .She left against medical advise,but she used oral hematinics for 6 months .

Later she lost follow up and she never visited hospital again .Her symotoms of bleeding PR ,jaundice fever subsided and never recurred according to patient.

After 3 years - she presented again with history of generalised weakness.On investigations her hemoglobin was still low - It was 3.3 ,TLC count was 4000 ,plt count -54000.She was treated as megaloblastic anemia .She recived 2 PRBC transfusion during this admission and hemoglobin improved to 6.5 and she got discharged .She had not used any IM or oral medicines.Later she continued to have fatigue , generalised weakness but she never came back for followup.

One year back because of persistent symptoms she got CBP done in private lab and was told to have severe anemia with thrombocytopenia.

Patient denies history of loose stools ,steatorrhea ,pain abdomen ,Malena ,hematuria,no night sweats ,no fevers ,no chronic cough 

Dietary history - She consumes mixed diet .She eats thrice daily .Her diet consist of fresh vegetables ,and twice monthly meat.

Since 1 month - she is been complaining of loss of appetite and weight loss.

Chief complaints - Patient this time presented to us with history of loss of appetite and loss of weight since 1 month.

Patient was amennorheic for 4 months , later she developed menorrhagia with hypermenorrhia - she bled for 10 days continuously - changing  3-4pads per day with large clots .She thought it would resolve but it was continuous and she fell ill and brought to our casualty with giddiness and profound fatigue and weakness.

On examination she was tachypneic ,pallor with bilateral pitting pedal edema ,Raised JVP.

Hyperpigmented knuckles ,

Bald tongue with loss of papillae.

BP-90/60 mmHg 

CVS - examination revealed normal apex impulse .Pansystolic murmur in aortic area - functional hemic murmur.

Other system examination was normal .

Investigations reavled - 

HB -1.9 ,TLc -4800 ,plt count - 80000

Diagnosis - 

1)Severe recurrent Bicytopenia with High output cardiac failure.

Peripheral smear suggest Megaloblastic anemia secondary to either nutritional or bone marrow failure syndomes .

Mennorhgia secondary to thrombocytopenia ? 

2)Hypoproteinemia - secondary to malnutrition / protein losing ? 

3) Subclinical Hypothyroidism

Discussion-

1) Despite the fact that patient recived multiple transfusions her Hemoglobin improved acutely but she recurred - 

It could be either blood loss or impaired bone marrow production.

2) Though she gives history of hemorrhoids 8 years back ,she never complained of Malena or bleeding per rectum after that episode .Stool for occult came out be negative .So anemia due to chronic blood less is less likely.

3) Decreased production is evident with low retic count and reticulocyte production index.

4) Impaired production can be either due to - Nutritional deficiencies ,bone marrow failure syndromes.

5) Marcovalocytes and hypersegmented neutrophils can be seen in either of the conditions.

6) Hypoalbuminemia , Hypoproteinemia - is it secondary to losing protien ? Or malnutrition ? 

As her CRP is negative and she doesn't have any acute febrile illness episode , Hypoalbuminenia as negative phase reactant is ruled out .

Approach to Bicytopenia/ pancytopenia -With megaloblastic picture: 

 

Ref:https://oncohemakey.com/approach-to-the-diagnosis-and-classification-of-blood-cell-disorders/

Etiology-

VITAMIN B12 pathway :

Ref: Shipton, Michael & Thachil, Jecko. (2015). Vitamin B12 deficiency - A 21st century perspective. Clinical medicine (London, England). 15. 145-50. 10.7861/clinmedicine.15-2-145. 

Tests for pernicious anaemia :To distinguish between pernicious anaemia and other causes of low cobalamin levels, it is necessary to test for serum autoantibodies.5 The anti-IF antibody test is a relatively insensitive (50–70%) yet highly specifi c (>95%) investigation;5 in contrast, the anti-PC antibody test has a sensitivity of >90% but a specifi city of only 50%.