A 14 year old girl was brought to the ER of a tertiary care hospital because she had fainted. On arrival her blood pressure was 90/50 mm Hg. Her pulse was 90 beats/min. She was conscious and alert. She was thin, afebrile and anemic. Her lymph nodes were not enlarged, the spleen was palpable but details could not be made out as the abdomen was “stiff”. The lungs were clear, the heart was in sinus rhythm and both heart sounds were audible and there were no signs of heart failure. She had been in the hospital for four weeks and had been feeling tired and fatigued for about a month. Her father was a tailor and earned about 80,000 rupees a month. The house they lived in had two bedrooms and there was access to electricity and water. There was access to a nutritious diet.She lived in a crowded suburb of Karachi.
This was the case presentation by the candidate.
What is your diagnosis?
Answer: Anemia of chronic illness.
What chronic illness does she have?
Answer: Pulmonary tuberculosis.
What evidence do you have for your diagnosis?
Answer TB is common in our part of the world. (Now you can see how this viva is headed for disaster). TB can be a silent disease. Anyway anemia is very prominent and she may have had fever.
At this stage the girl’s mother who was sitting in during the examination said ” I have been taking her to the doctor for the past 6 months. Her fever was checked and she had no fever whenever we visited the doctor’s morning clinic. I even had her chest X ray done but the doctor said it was clear.”
Would you like to talk to the patient and her mother and try and get to a diagnosis?
The mother elaborated that the girl had lost her appetite 6 months ago and had been living on a diet of 1/2 a chapatti a day with some soup. She ate no eggs, meat, chicken, yogurt and did not like milk. she avoided fruit as well. She had been losing weight, had visited the doctor in her area who did some tests, an X-ray chest but was unable to make a diagnosis. He had prescribed a bottle of vitamin syrup and an iron syrup but this caused her to have an upset tummy so she did not take these medicines. The diagnosis now became “iron deficiency anemia from a poor diet”.
Question: What caused her poor appetite? There was no satisfactory answer.
Question: Did she have any GI symptoms? The mother said that she had no nausea, vomiting or diarrhea. She had complained that her abdomen felt as if it was “full” all the time with a vague abdominal pain which she could not localize. Although she did not have a fever she felt hot and sweaty in the evening.
The examiner took another approach. What treatment was she given when she came to the hospital? The mother said she had received 4 blood transfusions. The candidate was asked if the transfusions had taken place rapidly or over a period of 6 months.
Question. As a matter of fact she was given 4 units of blood over a 48 hour period. What is the likely cause for the urgency to transfuse?
Answer: She had an active bleed. The candidate said that she must have excessive menses. (He had not inquired about her menarche etc before this. It transpired that she had had no menstrual periods for 5 months. It now seemed that we were going nowhere rather fast.)
Question. What are the indications for transfusions in anemia?
Answer. A hemoglobin of 7 Gm/dl.
A barrage of questions followed. Do you have to wait for the Hb to fall to 7 Gm before transfusing? If so why? What if the patient is in shock from whatever caused the low Hb? Would you wait for the Hb to fall in an active bleed or acute hemolysis? What level of Hb is maintained in thalasemia or chronic renal failure?
This patient actually had an Hb of 1.7 Gm/dL. This was the reason for treating her with blood transfusions.
Where this magic figure of 7 Gm has come from is a mystery but many candidates stick to it and fail in the examination without being able to explain that in post operative patients the mortality rate rises sharply as the Hb goes below 7 or 8 Gm so transfusion is a must. For an active bleed please do not wait until the Hb is 7 Gm. Transfuse early while monitoring the vital signs. Stable, asymptomatic patients can tolerate an Hb of 7 to 8 Gm but it is probably not wise to maintain such a low Hb. Cognitive functions decline below an Hb of 6 Gm in a patient with coma or a recent onset neurological deficit the correction of anemia rapidly is advisable.
For many decades, the decision to transfuse red blood cells (RBCs) was based upon the “10/30 rule”: transfusion was used to maintain a blood hemoglobin concentration above 10 g/dL (100 g/L) and a hematocrit above 30 percent. The 1988 National Institutes of Health Consensus Conference on Perioperative Red Blood Cell Transfusions suggested that no single criterion should be used as an indication for red cell component therapy, and that multiple factors related to the patient’s clinical status and oxygen delivery needs should be considered.
Oxygen delivery (DO2) is determined by the formula:
DO2 = cardiac output x arterial oxygen content.
Let us see how a pre-existing cardiac condition can be affected by anemia. Severe anemia causes high output failure and will make a pre-existing cardiac condition worse as in carditis, cardiomyopathy, valvular heart disease, ischemic heart disease. It can precipitate angina or even a heart attack.
Is the patient symptomatic? This girl had a syncopal attack. Cognitive functions: ability to reason and remember, decline with Hb below 6 Gm. Anemia can precipitate high output heart failure. Cardiovascular shock can occur if the patient is anemic because of blood loss, DIC (disseminated intravascular coagulation), septicemia.
Why was this girl anemic and can examination of her abdomen give us a clue. That she was emaciated due to a deficiency of calories was obvious. She could be iron deficient because she had restricted her diet. Why did she have such a poor appetite? Her abdomen was doughy to the touch and she had an enlarged spleen of which the tip was palpable. The right iliac fossa felt more full than the rest of her abdomen.
Did she have single nutrient deficiency? Iron for example. This is seen in malabsorption syndrome. She can have associated vitamin deficiencies such as riboflavin: angular stomatitis, glossitis, dermatitis around the nose, or folate deficiency or B12 deficiency. A careful examination for peripheral neuropathy and examination of the knee and ankle jerks is important. Beri beri can cause heart failure and polyneropathy.
If she has tuberculosis of the terminal ileum she could have B12 deficiency as well because this vitamin is absorbed from the terminal ileum. She needs tests to determine what type of anemia she has and imaging of her small intestines in particular to find out if she had TB intestines or not.
She was already improving, putting on weight with a month of anti-TB therapy and oral iron therapy.