Heam Flashcards

1
Q

Febrile Non-hemolytic Transfusion Reaction (FNHTR)

A

√ ↑ in Body Temperature of around 1 to 2 °C during or after blood transfusion.

√ Occurs during transfusion or up to 4 hours after transfusion.

√ Can present with Chills/ Rigors or Asymptomatic with ↑ Temperature only.

√ Rx: (Paracetamol and Monitoring)

Stop transfusion while continuing to give normal saline.

• Give Paracetamol.

• Resume the blood transfusion when the symptoms and fever subside.

• Observe the patient for 15-30 minutes

◙ FNHTR “Febrile Non-Haemolytic Transfusion Reaction”:

Fever of > 38

↑ in Temp. of at least 1 degree up to 2 degrees from pre-transfusion
temperature.

Other vitals are within normal with no significant changes from Pre-
transfusion vitals.

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2
Q
A
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3
Q

Acute Hemolytic Transfusion Reaction (AHTR)

A

AHTR can be fatal. It starts within minutes of transfusion. Some S&S include:

√ Fever,
√ Hypotension/ Shock,

√ Pain at the transfusion site
± DIC (↑ Bleeding),

± Haemoglobinuria/ Hemoglobinemia,
± Feeling of impending doom “death”.

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4
Q

CML

A

Chronic Myeloid Leukemia (CML)
• 40-50 YO.

• Massive Splenomegaly “May reach the right iliac fossa”.

Remember (CML = Crazy Massive Large Spleen)

• ↑↑↑ WBCs “often > 100 X 109/L “
• Differential shows Granulocytes at ALL STAGES of development without
Blast cells.

“↑ Neutrophils (Predominant), myelocytes, basophils, eosinophils”

• Cytogenetics → Ph Chromosome.

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5
Q

CLL

A

Chronic Lymphocytic Leukemia (CLL)

• Older (usually > 65 YO).

• Asymptomatic or may present with anemia and recurrent infections
(dysfunctional WBCs), lymphadenopathy …etc.

• ↑↑↑ WBCs (But dysfunctional) with B Lymphocytes Predominance.

• Blood smear → Mature Lymphocytes with Smudge cells.

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6
Q

ALL vs aplastic anaemia

A

Acute Lymphoblastic Leukemia (ALL)
• Children.

• Pancytopenia:
√ Low Hb → Anemia → Fatigue…etc.
√ Low WBCs → Recurrent Infections.
√ Lowe Platelets → Thrombocytopenia → Bleeding.

• Bone Marrow Aspiration/Biopsy → Numerous Blasts.

♦ In the exam, pancytopenia is either ALL or Aplastic Anemia. BM biopsy
can differentiate.

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7
Q

AML vs ALL

A

Acute Myeloid Leukemia (AML)
Rarely Asked. However, remember these simple points:

• ADULT (Not children)
• Auer rods on blood film. • Adults. • Gingivitis. • Gum bleeding

• Bone Marrow Aspiration/Biopsy → Numerous Blasts.

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8
Q

Numerous blasts!!

A

If you see numerous blast cells on Bone Marrow Biopsy and:

♦ The patient is a child → ALL (Acute Lymphoblastic Leukemia)

♦ The patient is an adult → AML (Acute Myeloid Leukemia).
Numerous Blasts = Acute Leukemia

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9
Q
A
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10
Q

A 25-year-old female arrives at the Emergency Department with a 6-day history
of worsening fatigue.

She also complains of persistent, watery diarrhoea that
started 5 days ago

. Over the last 48 hours, she has not passed any urine. Upon
examination, she is lethargic, has generalised oedema, and her blood pressure
is 135/85 mmHg.

Her heart rate is 88 beats per minute, and her respiratory
rate is 18 breaths per minute. Her abdomen is soft with normal bowel sounds

A

• The patient’s presentation suggests haemolytic uraemic syndrome (HUS),
which is often secondary to a gastrointestinal infection, commonly associatedwith Shiga toxin-producing Escherichia coli.

The classic triad of HUS includes
microangiopathic haemolytic anaemia, thrombocytopenia, and acute kidney
injury (AKI).

• Given the patient’s elevated potassium level (6.2 mmol/L) and evidence of
acute kidney injury, the most urgent and life-threatening issue to address is
hyperkalaemia.

• Dialysis is the most appropriate step to manage both the renal failure and
the dangerously high potassium level.
Summary of Key Considerations:

• Hyperkalaemia is life-threatening, especially at levels ≥6 mmol/L, and needs
immediate intervention to prevent fatal arrhythmias.

• Dialysis is indicated in severe AKI (acute kidney injury) with hyperkalaemia,
as it can efficiently remove excess potassium and manage fluid overload.

• Packed red cell transfusion (blood transfusion) may worsen hyperkalaemia
since stored blood contains potassium, which could exacerbate the patient’s
condition.

• Intravenous fluids are considered if there is a volume depletion cause of
AKI, but in this case, the patient shows signs of fluid overload.

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11
Q

Types of Dialysis:
√ Dialysis is A medical procedure that removes waste and excess fluid from the
blood when the kidneys cannot function properly.

√ Hemodialysis is the more common type of dialysis worldwide.

A

◙ Hemodialysis:
• Blood is taken from the body, filtered through a machine (dialyser), and
returned to the body.

• Requires a vascular access point (like a fistula or catheter).

• Typically performed in a hospital or dialysis centre.

Most common indications: Chronic kidney disease (CKD), acute kidney injury
(AKI) with severe electrolyte imbalances (e.g., hyperkalaemia),
fluid overload,

and uremia (build-up of waste products in the blood

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12
Q
A

◙ Peritoneal Dialysis:
• Uses the lining of the abdomen (the peritoneum) to filter the blood.
• A special fluid is placed in the abdomen, absorbs waste, and is then drained
out.

• Can be done at home, offering more flexibility.

• Most common indications:
CKD in patients who prefer home treatment, those

unable to tolerate hemodialysis,

or in certain cases of AKI

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13
Q
A
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14
Q

A 55-year-old man presents to his GP with a 2-month history of fatigue and
night sweats.

On physical examination, his spleen is found to be significantly
enlarged, extending 10 cm below the costal margin.

There is no
lymphadenopathy. Blood tests show a significantly raised white blood cell
count.

What is the most likely cell type that would be observed on a blood
smear?

A

• This presentation is highly suggestive of chronic myeloid leukaemia (CML),
which typically occurs in middle-aged to older adults.
• The patient’s symptoms of fatigue, night sweats, and massive splenomegaly,
combined with a markedly elevated white blood cell count, point towards CML.

• In CML, the blood smear characteristically shows a predominance of mature
granulocytes,

including neutrophils, basophils, and eosinophils, along with
their precursors.

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15
Q

Blast cells?

A

A) Blast cells: These are immature cells typically associated with acute
leukaemias.

In CML, blast cells are usually less prominent unless the disease
has progressed to a blast crisis, which is a more advanced stage.

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16
Q

Target cells?

A

B) Target cells: These are red blood cells with a bullseye appearance,
commonly seen in conditions

like thalassaemia
or liver disease.

They are not
associated with elevated white blood cell counts.

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17
Q

Helmet cells? Or schistocytes?

A

C) Helmet-shaped cells: These cells, also known as schistocytes, are
fragmented red blood cells seen in conditions like

haemolytic anaemia or
microangiopathic processes.

They are not typically associated with leukaemia.

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18
Q

Sickle cells?

A

D) Sickle cells: These abnormal, crescent-shaped red blood cells are seen in
sickle cell disease, a haemoglobinopathy. They do not present with a raised
white blood cell count or splenomegaly of this magnitude.

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19
Q
A
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20
Q

CLL?
Age?
Features?

A

Chronic
Lymphocytic
Older Adults (Over
60 Years Old)

  • Typically, no or mild splenomegaly.
  • Presence of smudge cells (fragile
    lymphocytes) in the blood smear
    (characteristic feature).
    Leukemia (CLL)
  • Cervical lymphadenopathy (enlarged lymph
    nodes in the neck).
  • Presence of mature lymphocytes (hallmark).
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21
Q

Chronic Myeloid
Leukemia (CML)

A

Massive splenomegaly (characteristic
feature, especially in later stages).
Middle Age (40-50)

  • Presence of the Philadelphia chromosome
    (BCR-ABL fusion gene) (hallmark).
    Chronic Myeloid
    Leukemia (CML)
    Years Old), but can
    occur in younger or
    older adults
  • Increased number of mature granulocytes
    (neutrophils, basophils, eosinophils) without
    blast cells.
  • Cells in all stages of maturation (e.g.,
    myelocytes, metamyelocytes, etc.).
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22
Q

Acute Myeloid
Leukemia (AML)
Adults (Common in
two age frames: 20-
40 Years Old and
Over 60 Years Old

A

Auer rods (needle-like inclusions) present in
the cytoplasm of myeloblasts (characteristic
feature).

  • Presence of immature blast cells in the bone
    marrow and peripheral blood (hallmark).
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23
Q

Acute
Children (e.g., Up to
Lymphoblastic
15 Years Old)
Leukemia (ALL)

A

Children (e.g., Up to
15 Years Old)

Pancytopenia (a reduction in the number of
red blood cells, white blood cells, and
platelets).

  • Presence of immature blast cells in the bone
    marrow and peripheral blood (hallmark).
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24
Q
A
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25
A man presents with 2 months history of fever, night sweats, and unexplained weight loss. He has splenomegaly. He has a history of visiting USA, New York City 2 months ago:
particularly: Non-Hodgkin’s lymphoma). The symptoms resemble that of brucellosis. However, the history of travel to New York City makes brucellosis less likely. Brucellosis develops after a history of consuming unpasteurised dairy products or direct contact with livestock. Brucellosis is extremely rare in urban areas like New York City due to effective public health measures and animal control programs.
26
particularly: Non-Hodgkin’s lymphoma). The symptoms resemble that of brucellosis. However, the history of travel to New York City makes brucellosis less likely. Brucellosis develops after a history of consuming unpasteurised dairy products or direct contact with livestock. Brucellosis is extremely rare in urban areas like New York City due to effective public health measures and animal control programs.
27
A man on apixaban (DOAC) for atrial fibrillation develops intracranial hemorrhage confirmed on CT scan.
→ Prothrombin complex concentrate.
28
◙ After initiating chemotherapy in a leukemia patient, he develops fatigue, lower back pain, decreased urine output, ↑ serum potassium, and deteriorated renal function test:
Tumor lysis syndrome (TLS). √ Remember: in TLS: ↑ Uric Acid, ↑ K+, ↑ Phosphate, ↓ Calcium. √ The most specific one of the above → ↑ Urate (uric acid) serum level √ TLS occurs mainly in Leukemia (Especially ALL) and Lymphoma (Particularly Burkitt’s Lymphoma) after initiating Chemotherapy. √ It is a hemato-oncological emergency as it rapidly leads to Renal Failure (High Urea and Creatinine). √ The management is complicated; however, remember that IV fluid is an important part of the management.
29
◙ Sickle cell disease patient + develop chest pain, fever, severe vertebral pain:
→ Admit for IV fluids, analgesics, and further investigations. Another valid answer → (Refer to hospital for same day review). (Acute sickle cell crisis, potentially complicated by acute chest syndrome even if the chest X-ray is clear. The vertebral pain is mostly due to ischemic crisis seen in sickle cell patients. Remember that sickled RBCs can obstruct vessels in various tissues causing ischemia eg, bone ischemia and infarction)
30
A 55-year-old man has recently -4 days ago- had intracerebral haemorrhage. Now, he developed DVT in the left leg confirmed by doppler U/S “large thrombus extending from the common femoral vein up into the iliac vein”. What is the most appropriate management?
→ Percutaneous thrombectomy. • Note that in a recent hemorrhagic stroke, it is contraindicated to give anticoagulants eg, Low molecular weight heparin (LMWH), Direct Oral Anticoagulants (DOAC), Unfractionated heparin, Warfarin
31
A 58-year-old man underwent a gastric banding 18 months ago presents with lethargy, limb weakness, and paraesthesia of fingers. What to request?
Performing gastrectomy → Reduces the gastric intrinsic factors → Reduces vitamin B12 absorption → Results in Vitamin B12 deficiency → Which can manifest as limb weakness, paraesthesia, and lethargy due to anemia. (Haematinics = vitamin B12, Iron, Ferritin and → Request Haematinics Folate).
32
• The presence of: fatigue, bone pain, recurrent infections, anemia, hypercalcemia (↑Ca++), monoclonal spike on protein electrophoresis suggest multiple myeloma. • Multiple myeloma is a malignancy of plasma cells.
→ Urgent referral to a haematologist (within 2 weeks). • in hemato-oncology → confirmation by bone marrow biopsy → Staging of the disease → Initiation of the treatment.
33
If a multiple myeloma patient presents to the GP with a sudden severe back pain
→ suspect spinal cord compression → Send immediately to A&E to be seen and evaluated by orthopaedics. Spinal cord compression is serious and could result in neurological damage. A GP would not be able to deal with it.
34
The sites of the main absorption of:fe Folate VIT b12
• Iron → Duodenum. • Folic Acid (Folate) → Duodenum and Jejunum. • Vit B12 → Terminal (Distal) Ileum. • Bile salts → Terminal (Distal) Ileum. • The majority of nutrients → Jejunum. To make it easy, memorise the following: Iron → duodenum. Folate → jejunum (and duodenum). Vit B12 → Terminal ileum.
35
Q1) A man with folate deficiency. What is the affected organ (if any)?
→ Duodenum/ jejunum (proximal small intestine) eg, crohn’s, celiac.
36
Q2) Ileal resection (malabsorption) + Fatigue and Palpitation (due to Anemia) + low hemoglobin and High MCV. What is the deficiency?
→ Vitamin B12 deficiency. Low Hb and high MCV → megaloblastic (vit. B12 OR Folate deficiency). Ileal resection? Vitamin B12 is absorbed mainly in ileum, while folate is absorbed mainly in duodenum and jejunum. So → Vit. B12 deficiency.
37
Infection (sepsis: high temperature, high WBCs, high CPR, high heart rate) + Brusing, purpura, bleeding from sites: eg, venepuncture sites, nose (epistaxis) + ↑ PT, ↑ PTT, ↓ Platelets.
Think → Disseminated Intravascular Coagulopathy (DIC).
38
young man presents with several attacks of pancreatitis. He has peripheral paraesthesia, loss of proprioception in his legs, loss of memory and difficulties with thinking.?
Dx→ Vitamin B12 Deficiency. Rx → Hydroxocobalamin (ie, vitamin B12 IM injections).
39
A man has low serum folate and low vitamin B12 which to treat first?
→ Treat vitamin B12 deficiency first (eg, give vitamin B12 injections). (Mnemonic: B before F → treat vit B12 deficiency before Folate deficiency)
40
A man has low serum folate?
→ Encourage him to eat leafy green vegetables.
41
Vitamin B12 Deficiency “updated” Megaloblastic Anemia = low Hb, high MCV → Either vitamin B12 deficiency or Folic acid deficiency or both. Vitamin B12 = Cobalamin. Causes of b12 def??
◙ Causes of Vitamin B12 deficiency → Loss of intrinsic factors - Pernicious Anemia (The most common cause). Pernicious Anemia → Autoimmune Gastric Atrophy that are required for Vit B12 absorption. Usually associated with other autoimmune diseases eg, hypothyroidism. - Total Gastrectomy (Impaired Vit B12 Absorption). - Ileal Resection. (Malabsorption: the majority of vit B12 is absorbed in the terminal ileum) - Crohn’s Disease. - Chronic Pancreatitis (malabsorption). - Celiac Disease (malabsorption). - Dietary (Vegans). Remember that Vit B12 is present in meat, fish and dairy products but not in the vegetables. Folic acid is in green vegetables. Thus, vegans often develop vitamin B12 deficiency. And, those who do not eat vegetables often develop Folate deficiency.
42
◙ Features of Vitamin B12 deficiency
◙ Features of Vitamin B12 deficiency - Peripheral paraesthesia. - Impaired position and vibration (proprioception) sense. - Dementia → loss of memory + difficulties with thinking. - If untreated → permanent Ataxia.
43
Lab findings of b12 def?
◙ Lab Results of Vitamin B12 deficiency - ↑ MCV (usually > 110) + ↓ Hb: Macrocytic Anemia. - Hypersegmented Neutrophils on a blood smear. - ↑ Homocysteine.
44
These Lab results are also present in Folic Acid deficiency. So, How to Differentiate?
By the History: - Vegans (who do not eat meat, fish, dairy products) → Vit B12 deficiency. - If the patient does not eat vegetables → Folic Acid deficiency. - Gastric or ileal resection → Pernicious Anemia → Vit B12 Deficiency. ◙ Treatment of Vitamin B12 Deficiency → IM Hydroxocobalamin (ie, Vitamin B12 IM injections
45
◙ If INR < 5 (but still higher than the target level
): √ Reduce (Or) Omit one or two doses of Warfarin. √ Measure INR in 2-3 days.
46
◙ If INR 5-8 + NO Bleeding:
√ Stop Warfarin and Check INR the following day. √ Restart warfarin when INR < 5. (Similar to the above one but here no bleeding and so no vitamin K). So, if the INR between 5 and 8, stop warfarin and restart it when INR is < 5. (This is in all cases). If WITH minor bleeding (eg, epistaxis), give vitamin K. If No bleeding, no need for vitamin K.
47
◙ If INR 5-8 + Minor Bleeding
: √ Stop Warfarin and Check INR the following day. √ Administer IV or oral Vitamin K1 (Phytomenadione). √ Restart warfarin when INR < 5.
48
◙ If INR is > 8 ± Minor Bleeding:
√ Stop Warfarin. √ Administer IV or oral Vitamin K1 (Phytomenadione). √ Restart warfarin when INR < 5.
49
◙ If it is a MAJOR bleeding and the patient is on warfarin:
√ Stop warfarin. √ Give IV 5 mg vitamin K1 (to reverse warfarin. Careful: IV NOT ORAL)! √ Give prothrombin complex concentrate (PCC) or if unavailable: fresh frozen plasma -FFP-). If you are to pick between (PCC) and (Vit. K) in the exam, pick → PCC. This is because PCC works faster. In reality, we would give both in major bleeding
50
Q) Why is it important to receive parenteral vitamin B12 after gastrectomy?
→ Due to loss of intrinsic factors. Gastrectomy → Loss of the intrinsic factors that are produced from stomach → impaired Vitamin B12 (Cobalamin) absorption → Vitamin B12 deficiency. Parenteral vitamin B12 = IM Hydroxocobalamin. Stomach produces intrinsic factors that help the absorption of vitamin B12 later in small intestine and distal ileum.
51
• [After surgery], if the patient will be immobile for a while, it is beneficial to_____ And after acute stroke_____
to prevent DVT. • If the patient is admitted with [Acute Stroke], to prevent DVT, use: → Intermittent pneumatic compression. Be careful, intermittent pneumatic compression is preferred if the patient is admitted with acute stroke! Elastic compression stockings are not beneficialin acute stroke; they are helpful in general surgeries. Both are used to prevent venous thromboembolism. Both were asked before.
52
When is Blood Transfusion Indicated?
• If Hb < 70 g/L (either symptomatic or not). • If Hb < 80 g/L (+) symptoms of anemia (eg, fatigue, tachycardia, pallor). • If Hb < 90 g/L (+) there is a known cardiovascular disease. Very low hemoglobin puts patients at risk of arrhythmia and heart failure.
53
A 35 YO woman with history of menorrhagia presented with fatigue. Her MCV is low and her hemoglobin is 68 g/L. What should be done? (or) (or) RBC transfusion IV iron supplements Oral ferrous sulphate?
Rbc transfusion • Her low MCV and low Hb + Hx of menorrhagia → Iron deficiency anemia. • Since Hb <70 g/L → (she is also symptomatic; fatigued)! Note: 70 g/L is the same as 7 g/dL (but the unit is different).
54
55
A 61 YO man presents for medication review. He has a history of ischemic stroke a few months ago. His medical background includes hypertension and type 2 diabetes mellitus. His ECG now shows arterial fibrillation. He is on clopidogrel, amlodipine, atorvastatin, and metformin. What is the most appropriate action? ? A) Switch amlodipine to ramipril. B) Continue clopidogrel and add apixaban. C) Stop clopidogrel and start edoxaban. D) Continue amlodipine and add ramipril. E) Continue amlodipine and add bendroflumethiazide.
The right answer is ©: Stop clopidogrel and start a DOAC eg, edoxaban. • He did not have atrial fibrillation before, that’s why he was on clopidogrel (and not warfarin or DOAC). • Since he is now having atrial fibrillation, switch clopidogrel to an anticoagulant such as warfarin or DOAC (eg, edoxaban, apixaban). DOAC is preferred over warfarin. • His blood pressure is not mentioned so we assume it is controlled and therefore no need to add an additional antihypertensive (even if he is diabetic, he is already on amlodipine and controlled).
56
A 65 YO man presents for medication review. He has a history of ischemic stroke a few months ago. His medical background includes atrial fibrillation, hypertension and type 2 diabetes mellitus. His ECG still shows arterial fibrillation. He is on warfarin, lercanidipine (CCB), atorvastatin, and metformin. His current blood pressure is 160/100. What is the most appropriate action? A) Switch warfarin to Edoxaban (DOAC). B) Continue warfarin and add clopidogrel. C) Stop lercanidipine and start ramipril. D) Continue lercanidipine and add ramipril. E) Continue lercanidipine and add bendroflumethiazide.
The right answer is (D): continue lercanidipine (CCB) and add ramipril (ACEi). • Since he is diabetic, ACEi is of choice to control HTN. • Since he is already on an antihypertensive (which is lercanidipine; a calcium channel blocker) and his hypertension is still uncontrolled, go to step 2 and add a second antihypertensive (ACE inhibitor eg, ramipril). • We do not shift from warfarin to DOAC unless his INR is not normal. Here, no mention of INR. Thus, continue on warfarin (option A is wrong). • Since he has AF, an anticoagulant (eg, warfarin, DOAC) is given; Not an antiplatelet (eg, clopidogrel).
57
√ Fatigue, Tiredness, Lethargy (+) √ Pruritus “itching” especially after hot shower (+) √ Splenomegaly (+) √ Burning sensations in fingers and toes (+) √ Gout “due to ↑ cell turnover”
→ Polycythemia Rubra Vera (PRV) “= Primary Polycythemia” • It is a malignancy. • Mutation in JAK2 gene → JAK Mutation Screen is the Investigation of Choice. → Excessive Proliferation of RBCs, WBCs and Platelets (All ↑) → High “Hematocrit”: may exceed 55% Hyper viscosity of the blood → ↑ Risk for MI, DVT, PE, Stroke.
58
• Other manifestations polycýthenia Vera rubrA √ Facial Plethora “redness”. √ Headache √ Hepatomegaly/ Splenomegaly √ Low/normal serum erythropoietin. [Note: In Secondary Polycythemia → Erythropoietin is High]. RX?
• Treatment: √ Venesection “Phlebotomy” → to reduce the elevated haematocrit and thus reduce the blood viscosity “the mainstay step of the treatment”. √ Low dose Aspirin (75 mg OD) → for fear of thrombosis. √ It is a malignancy → Chemotherapy: < 40 YO → Interferon ▐ 40 YO → Hydroxyurea.
59
Secondary polycythemia- 2ndary erythrocytosis
♦ Remember in Polycythemia Rubra Vera “PRV” (Primary Polycythemia), all 3 lines: Hemoglobin, WBCs and Platelets are Elevated. ♦ In Secondary Polycythemia, Only Hemoglobin is Elevated. ♠ One important cause of 2ry Polycythemia to remember is Chronic Hypoxia (such as those who are chronic smokers → COPD). Other causes → High Altitudes/ Cyanotic Congenital Heart diseases. ♠ Chronic Hypoxia → Stimulates the kidneys to produce more and more Erythropoietin → Which stimulates the bone Marrow to produce → More and more RBCs so they can carry more O2 to the tissue
60
Difference btw polycythemia primary and secondary
Important note √ In Primary Polycythemia (PRV) → Erythropoietin is either Low or Normal. √ In Secondary Polycythemia → Erythropoietin is High
61
Glucose-6-Phosphate-Dehydrogenadse (G6PD) Deficiency Risk factors Cause Features
◙ ↓ G6PD enzyme → ↓ Glutathione → RBCs become vulnerable to oxidative damage → Hemolysis. ◙ Exposure to precipitating factors such as certain drugs “ e.g. anti-malarial Primaquine”, Fava beans, Infection → Severe Hemolysis → Jaundice, Red or Dark Urine, Back and Abdominal pain. ◙ Important Triggers [useful in the exam]: Fava Beans ▐ Anti-malarial drugs ▐ Sulfa drugs ▐ Infections (e.g. UTIs)
62
Important triggers and treatment of g6pd def Pay Attention! Sometimes the question won’t have G6PD deficiency in the options. Instead, pick (Hemolytic Anemia).
◙ Important hints towards G6PD Deficiency in the exam: √ Male (as the disease is X-linked Recessive) √ African or Mediterranean. √ Heinz Bodies or Bite Cells. √ Recent infection or recent ingestion of drugs or fava beans. √ Hx of Neonatal Jaundice. √ Presents with one or more of: Jaundice, Dark urine , Back and Abdominal pain. ◙ The definitive Diagnostic Test → G6PD enzyme activity (6 weeks after the hemolytic attack). ◙ Treatment: • Avoid Triggers. • IV fluids. • If severe Haemolysis → Blood Transfusion.
63
64
What test is used in cross-matching “Blood Transfusion Preparation”?
Answer → Indirect Coomb Test (Indirect Anti-Globulin Test)
65
Direct and indirect Coombs test
◙ Direct Coomb Test (Direct Anti-globulin test): √ It detects antibodies on the RBCs Surfaces. √ Used for → Autoimmune Haemolytic Anemia (AHA). ◙ Indirect Coomb Test (Indirect Anti-globulin test): √ It detects antibodies in the Serum. √ 2 Major uses: • Blood Transfusion Preparation (Cross-matching). • Antenatal antibody screening → Screening a pregnant ♀ for IgG antibodies that can cross the placenta and cause hemolysis in fetal blood.
66
◙ Osmotic Fragility test → ______
◙ Osmotic Fragility test → Hereditary Spherocytosis.
67
A 57-year-old man presents with lethargy and he looks pale. Blood pressure is 150/100. Urinalysis: Blood +++, Protein +++, Creatinine: Elevated, Calcium is high Other tests are normal. What’s the appropriate investigation among the following options?
Pale and lethargy → Anemia Renal Failure Hypercalcemia Others (not mentioned here): Bone pain, Recurrent infection Think → Multiple Myeloma ◙ Urine electrophoresis → Bence Jones Protein. ◙ The cell type to be found in BM → ◙ plasma cells The Diagnostic Test → ◙ BM biopsy The likely finding on blood film →rouleax formation Bone Marrow Biopsy. Rouleaux Formation.
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A patient present with Pancytopenia (Low Hb, Low WBCs, Low Platelets). What is the diagnosing test?
→ Bone Marrow Biopsy Some Important Differentials for Pancytopenia: √ Acute Lymphocytic Leukemia → Numerous Blasts. √ Aplastic Anemia → Hypoplastic bone marrow. (Reduction in hemopoietic cells)
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◙ Hypercalcemia picture e.g. ↑ Thirst “Polydipsia”, Polyuria, bone pain, confusion, low mood Causes
d) Think of: • Bone metastasis “e.g. from prostate, breast”. • SCC of the lung. • Multiple Myeloma. (ALP is normal) • Primary Hyperparathyroidism (associated ↑ PTH, ↓ Phosphate). ◙ Hypercalcemia picture: • Neuro → lethargy, Confusion, Depression. • GIT → Constipation. • Renal → polyuria (increased urination), Polydipsia (Thirst). • CVS → ECG: Short QT interval
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Low Hb, Low MCV, Low Ferritin, low transferrin saturation High TIBC High RDW (total iron binding capacity) (red cell distribution width)
→ Iron Deficiency Anemia Other features of IDA: √ Angular Stomatitis (can also occur with Vit B12 def., look at MCV). √ Red sore tongue √ Koilonychia Causes of IDA: ◙ The most common cause → GIT Blood Loss → e.g., NSAIDs use, Colorectal Carcinoma, Gastric Carcinoma, Gastric or Duodenal Ulcer. ◙ The most common cause in premenopausal ♀ → Menorrhagia. ◙ Other causes: • Dietary inadequacy. • ↑ iron requirements → Pregnancy. • Malabsorption → e.g., Celiac disease.
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RX of fe def anaemia
Treatment: √ Treat the underlying cause. √ Iron supplements (e.g., ferrous sulphate). When is Blood Transfusion indicated? • If Hb < 7 (either symptomatic or not). • If Hb < 8 + there are symptoms of anemia (eg, fatigue, tachycardia, pallor). • If Hb < 9 + there is a known cardiovascular disease.
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An old lady with Rheumatoid Arthritis (RA) is on Methotrexate found to have anemia (low Hb and low MCV).
√ Both Vitamin B12 and Folate deficiency have Macrocytic anemia (high MCV), here, it is low. Although methotrexate is anti-folic acid, the MCV here is low. √ No Hx of menorrhagia is mentioned. Plus, she is old, and likely postmenopausal √ Although Anemia of chronic disease is usually Normochromic Normocytic, it can be hypochromic microcytic such as in the cases of Rheumatoid Arthritis and Crohn’s disease.
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A woman presents complaining of fatigue and heavy menstrual bleeds. Her Hb is 88 g/L (Low) and MCV is 67 (low).
Oral Ferrous Sulphate. The most appropriate management → ferrous sulphate oral She has Iron deficiency Anemia 2ry to menorrhagia. We give oral iron supplement.
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Henoch-Schönlein Purpura (HSP)
• Purpura is non-blanching and mainly on the buttocks and Lower Limbs. • Precipitated by URTI – Sore Throat. • All Blood Results are NORMAL “Normal Hb, WBCs and Platelets”. Others: • Arthralgia “Esp. Knee”. • ↑ ESR, ↑ IgA. • Rarely, • Rx → Self-limiting (e.g. Aspirin for arthralgia Unless there is renal impairment).
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Hsp
HSP → PAAN: Purpura, Arthralgia, Abdominal pain, Nephropathy “not always” (Hematuria, Proteinuria).
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Example (1), A 10 YO boy presents with a low-grade fever and macular rash especially on the back of the legs following an upper respiratory tract infection. A few hours later, these macules have turned into purpuric lesions that do not blanch on glass test. The boy also complains of joint stiffness and headache. Hb (124 g/L) ▐ WBC (3.3 X 109/L) ▐ Platelets (219 X 109/L).
The Likely Dx → Henoch-Schonlein Purpura. Note that Hb, WBCs, and platelets are normal. Please note that if a patient presents with similar features but with LOW PLATELETS and normal Hb and WBCs, the answer would be → Idiopathic Thrombocytopenic Purpura
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Example (2), 17 YO boy presents with palpable rash on his buttocks and extensor surfaces of his arms and legs that started following sore throat. He also has crampy abdominal pain, joint stiffness and joint pain. Urine Testing reveals microscopic hematuria and proteinuria.
The Likely Dx → Henoch-Schonlein Purpura.
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14 YO male presents with mucosal bleeding, petechial rashes, tiredness and pallor. Hb 77 ▐ WBCs 1.8 ▐ Platelets 30 ▐ Neutrophils 0. Blood Film shows Unremarkable “Normal” Morphology. Absent Reticulocytes. Bone Marrow Aspirate shows a marked reduction in all haemopoietic tissues replaced by fat spaces.
The likely Dx → Aplastic Anemia. Pancytopenia = ↓ Hb, ↓ WBCs, ↓ Platelets ♦ In the exam, pancytopenia is either Acute Lymphocytic Leukemia or Aplastic Anemia. BM biopsy can differentiate. BM Aspirate/ Biopsy → Numerous Blasts → Acute Lymphocytic Leukemia “ALL” BM Aspirate/ Biopsy → Reduction in all haemopoietic tissues replaced by fats spaces → Aplastic Anemia.
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Hairy Leukoplakia
→ Irregular folded or ridged white patches on the sides of the tongue. HIV disease → Supportive of a Dx of → EBV invades the Weak Immune System (e.g. HIV patients) and causes this lesion. → Cannot be scraped off. → It is benign and needs no treatment
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A 42 YO ♂ presents with mild fever, tiredness, marked weight loss recently and bilateral white corrugated lesions on the lateral surfaces of his tongue.
The likely Dx → HIV Disease.
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Target inr
The target INR in most cases “Including Warfarin intake for AF, DVT” → 2-3 ◙ The target INR in mechanical valve replacement “Metallic Valve” → 3-4
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50 YO ♂ complains of headache, pruritus, Hx of DVT. Hb: 190 g/L▐ WBCs: 15 X 109/L ▐ Platelets: 802 X 109/L.
The likely Dx → The Main Treatment line → PRV “Polycythemia Rubra Vera” Venesection “Phlebotomy” In PRV: √ Pruritus (Especially after hot shower) ▐ Gout ▐ Burning sensation of fingers and toes▐ Splenomegaly ▐ Headache … etc √ All three cell lines are elevated (High Hb, WBCs and Platelets). √ Low/normal serum erythropoietin. √ hyper viscosity ► High risk of DVT, Pulmonary embolism…e
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Idiopathic Thrombocytopenic Purpura (ITP)
• Mainly affects children, but can occur in adults. • In children, it is usually proceeded by infection. (Low Platelets but other labs are normal). • Bleeding, epistaxis, petechia, menorrhagia. Sometimes Asymptomatic. • Isolated Thrombocytopenia • Rx → √ Prednisolone, √ IV Ig (IV immunoglobulins). √ Life-threatening hemorrhage(platelets < 20) → Emergency Platelet Transfusion. If you see Isolated Thrombocytopenia ± Bleeding → Think of ITP.
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Itp
• Mainly affects children, but can occur in adults. • In children, it is usually proceeded by infection. (Low Platelets but other labs are normal). • Bleeding, epistaxis, petechia, menorrhagia. Sometimes Asymptomatic. • Isolated Thrombocytopenia • Rx → √ Prednisolone, √ IV Ig (IV immunoglobulins). √ Life-threatening hemorrhage (platelets < 20) → Emergency Platelet Transfusion. If you see Isolated Thrombocytopenia ± Bleeding → Think of ITP.
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Normal PT: 10-14 sec. Normal PTT: 35-45 sec. Bleeding Time: 3-9 minutes.
↑ PTT + (Bleeding into muscles or joints or easily bleeding) → think of haemophilia. • ↑ PTT and ↑ Bleeding Time + (Mucosal Bleeding) → think of VWD “Von Willebrand Disease”. • ↑ PTT and ↑ PT and ↑ Bleeding Time + (Bleeding at any site e.g. purpura, petechia, GIT, ENT, venepuncture site) → think of DIC. Others: ↓ Platelets and fibrinogen ▐ ↑ D-dimer)
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14 YO ♂ has developed excessive bleeding after tooth extraction. He notes that he bruises easily with mild trauma. Hb 120 g/L ▐ WBC 7 ▐ PT 12 ▐ PTT 84 ▐ Bleeding time: normal
◙ The likely Dx → Haemophilia. ◙ Haemophilia A or B → Haemophilia A (More common). Isolated Prolonged PTT → Hemophilia.
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Difference between haemophilia A and B
Hemophilia A Hemophilia B = “Christmas Disease” More common (90% of the cases) Less common -Factor VIII (8) deficiency. Factor IX (9) deficiency. Rx: Rx: √ Desmopressin (it increases Factor 8) √ Desmopressin has no role. √ Major bleeding → Recombinant factor 8 √ Recombinant factor IX (of choice). Important: DO NOT give NSAIDs or IM injections in hemophilia (↑ bleeding)
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◙ Notes: • ↑ PTT + Normal PT and Bleeding Time + (Bleeding into muscles or joints or easily bleeding or after tooth extraction) → think of haemophilia. • Haemophilia is X-linked recessive → mainly affects Males. • Important X-linked recessive conditions → G6PD def., DMD, Hemophilia. • Important: Hemophilia B is also called → Christmas Disease
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6 YO boy, recurrent attacks of hemarthrosis (bleeding into knee and elbow joints). Factor VIII/XI assay shows low factor VIII.
The likely Dx → hemophilia A The most appropriate treatment → Desmopressin.
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40 YO ♀ presents with tiredness that started 2 weeks ago. She is mildly jaundiced. HR 78, BP 120/70. Labs are as follow: Hb 92 g/L ▐ MCV 98 fL (high, the normal range is : 76-96) ▐ WBCs 8 ▐ Bilirubin 39 (high) ▐ ALT, AST, ALP, Gamma Glutamyl Transferase: normal.
◙ The appropriate Investigation → Direct Antiglobulin (Direct Coomb) Test. Here we have Anemia + High MCV + High Bilirubin → there is hemolysis! Anemia + High Bilirubin = Hemolysis What about the High MCV? We know that macrocytic anemia (Folate and Vit. B12 Deficiency) shows high MCV; however, hemolytic anemia can sometimes show high MCV. Markers of Hemolysis: ↑ LDH, ↑ Indirect Bilirubin “Jaundice”, ↑ Reticulocytosis, Sometimes ↑ MCV and MCHC indicate hemolysis.
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Direct and indirect Coombs test
Direct Antiglobulin (= Direct Coomb) Test → Autoimmune Haemolytic Anemia. Indirect Antiglobulin (= Indirect Coomb) Test → Cross-Matching (blood transfusion preparations) / Antenatal Screening.
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Markers of hemolysis
Markers of Hemolysis: ↑ LDH, ↑ Indirect Bilirubin “Jaundice ↑ Reticulocytosis, Sometimes ↑ MCV and MCHC indicate hemolysis.
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38 YO ♀ presents with tiredness. She is mildly jaundiced. Hx of URTI. Labs are as follow: Hb 92 g/L ▐ MCV 99 fL (normal: 76-96) ▐ WBCs (4-11) ▐ Bilirubin 29 (high) ALT, AST, ALP and Gamma Glutamyl Transferase are normal. Peripheral Blood Smear show → Polychromasia + Spherocytes.
• This is most likely is a case of • Markers of Hemolysis: Autoimmune hemolytic anemia. ↑ LDH, ↑ Indirect Bilirubin “Jaundice”, ↑ Reticulocytosis, Sometimes ↑ MCV and ↑MCHC indicate hemolysis as well. Request → Direct Coomb Test ▐ Also known as Direct Antiglobulin test.
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Hereditary spherocytosis and AIHA differences Hemolysis Anaemia Mcv elevated Bilirubin elevated
◙ In this scenario: Anemia + ↑ bilirubin (jaundice) + ↑ LDH + ↑ MCV and MCHC → Anemia with Hemolysis → Direct coomb (antiglobulin) test to confirm autoimmune hemolytic anemia. • Polychromasia = ↑↑↑ immature RBCs. • Spherocytes → Hereditary Spherocytosis? Nope! Not necessarily! Spherocytes can be seen in both Hereditary Spherocytosis and Autoimmune Hemolytic Anemia. To Differentiate → Direct Coomb Test. In this scenario, although we suspect hereditary spherocytosis, Direct Coomb “Direct antiglobulin” test would differentiate the cause of the hemolysis. This is because it will be +ve in Autoimmune Hemolytic Anemia, and -ve in Hereditary Spherocytosis. Osmotic Fragility Test is positive in both Hereditary Spherocytosis and Autoimmune hemolytic anemia. Direct coomb “antiglobulin” test is positive in Autoimmune hemolytic anemia and negative in Hereditary Spherocytosis. Thus, Direct coomb is more appropriate as it can differentiate the 2 cases.
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An elderly man with dementia, recurrent visits to the hospital for bruises in the face, head and forearms.
→ Suspect “non-accidental injury”. Someone is abusing this old man as the bruises are in suspicious sites. If the cause was “recurrent falls” for instance, the bruises would have been over the hip, knee or shoulder joints, not in the face! And not on several distant body areas!
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HELLP synd AFLD DIC
• HELLP Syndrome → Hemolysis (low Hb), Elevated Liver enzymes, Low Platelets. • Acute Fatty Liver of Pregnancy (AFLP) → ELLP (without Hemolysis) + (↓) Glucose ± (↑) Ammonia • Disseminated Intravascular Coagulation (DIC) → High PT, High PTT, High Bleeding Time, Low Platelets, Low Fibrinogen
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Example, A pregnant woman in her 35 weeks pregnancy developed sudden severe (acute) abdominal pain and is taken for emergency C-Section. Her BP 110/60. Labs: Hb: 101 ▐ WBC 9.5 (Normal) ▐ Platelets 65 (Low) ▐ PT 28 sec (high) ▐ PTT 67 sec (high) ▐ Fibrinogen 0.7 (low) ▐ Bilirubin 23 (high)
The likely Dx → Disseminated Intravascular Coagulation (DIC). High PT, PTT Low Platelets, Fibrinogen → DIC (see the comparison above) ◙ Remember: DIC Triggers→ sepsis, surgery, major trauma, cancer, and complications of pregnancy This lady might have developed Placenta abruption which has led to DIC.
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A 4 YO boy has easy bleeding into joints following a minor trauma. His uncle and grandfather had the same condition.
The likely mode of inheritance → X-linked. ◙ 2 ways to answer this question: This is likely a case of hemophilia which is X-linked recessive. (Bleeding into joints and muscles is common in Haemophilia -factor type bleeding-). 1 √ 2 √ The fact that all the affected individuals mentioned in the stem are males (the little boy, the uncle and the grandfather) makes it more likely to be X-linked.
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) A 16 YO girl presents with profuse bleeding after a dental extraction. Her father and paternal grandmother have experienced similar problems.
The likely mode of inheritance → Autosomal Dominant. √ This likely a case of VWD “Von-Willebrand Disease → Mucosal Bleeding + can show symptoms (The other X in Females can compensate for the affected X). NOT X-linked”. √ Most Cases of VWD are → Autosomal Dominant. √ This scenario CANNOT be X-linked as a girl and a grandmother (females) are affected. In X-linked diseases, nearly only males are affected or at least can show symptoms (The other X in Females can compensate for the affected X). In this scenario, the paternal grandmother has passed the condition to the father (male), and the father has passed it to his daughter (girl). A father with an X-linked condition has ZERO chance to inherit the condition to his Sons. However, 100% of his daughters will be carriers.
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◙ A Quick Recap: √ Hemophilia → X-linked Recessive. √ G6PD deficiency → X-linked Recessive. √ VWD → Mostly Autosomal Dominant. √ Hereditary Spherocytosis → Mostly Autosomal Dominant. √ Thalassemia → Autosomal Recessive. √ Sickle Cell Anemia → Autosomal Recessive.
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Inheritance AR AD X linked
◙ Autosomal Recessive Conditions: (25% if both parents are carriers) ◙ Autosomal Dominant Conditions: (50% if One parent is affected) ◙ X-linked Recessive Conditions: (Male: 50% if mother is carrier)
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Enzyme inducers and inhibitors
◙ Cytochrome P450 Enzyme Inducers → ↓ the anticoagulant effect of the Warfarin → ↓ INR. ◙ Cytochrome P450 Enzyme Inhibitors → ↑ the anticoagulant effect of the Warfarin → ↑ INR. Inducers → Decrease Inhibitors → Increase
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Enzyme inducers Crapgps
• Carbamazepine • Rifampin • Alcohol “Chronic” • Phenytoin • Griseofulvin • Phenobarbital • Sulphonylures
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Enzyme inhibitors Sick faces.com
Sodium Valproate. • Isoniazid. • Cimetidine. • Ketoconazole. • Fluconazole. • Alcohol (Acute drinking). • Chloramphenicol. • Erythromycin (Macrolides: Clarithromycin) • Sulfonamides. • Ciprofloxacin. • Omeprazole. • Metronidazole
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Enzyme inducers
If used with COCP, additional contraceptive method is needed (e.g. Depo-Provera, IUS, barrier methods) as these enzyme inducers weaken the COCP and PO Decreases Warfarin effect → ↓INR
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An elderly ♀ presents with chest infection and thus was started on clarithromycin. Her Hx includes, taking Carbamazepine for trigeminal neuralgia, taking Warfarin for mechanical valve replacement, taking Bisoprolol, Amlodipine and Atorvastatin. Her INR was found to be 1.4 (The target for mechanical valve replacement is 3-4). What is the causative drug for this low INR?
◙ Carbamazepine is a P450 enzyme inducer; thus, it will decrease the anticoagulant effect of Warfarin and therefore leads to ↓ INR. ◙ Clarithromycin is a Macrolide (like erythromycin). It is a P450 enzyme inhibitor and thus leads to increase the anticoagulant effect of Warfarin and therefore ↑ INR. ◙ The remaining drugs in the stem has no effect on P450 enzyme.
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Conditions AR AD Xlinked
√ Cystic Fibrosis, Congenital Adrenal Hyperplasia → Autosomal Recessive. √ ADPKD, BRCA gene, Neurofibromatosis, Huntington’s → Autosomal Dominant. √ Duchenne Muscular Dystrophy (DMD) → X-linked Recessive. • Alport Syndrome → X-linked (0% to pass from a father to a male child)
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An elderly ♀ presents with chest infection and thus was started with clarithromycin. Her Hx includes, taking Carbamazepine for trigeminal neuralgia, taking Warfarin for mechanical valve replacement, taking Bisoprolol, Amlodipine and Atorvastatin. Her INR was found to be 1.4 (The target for mechanical valve replacement is 3-4). What is the causative drug for this low INR
The answer → Carbamazepine ◙ Carbamazepine is P450 enzyme inducer; thus, it will decrease the anticoagulant effect of Warfarin and therefore leads to low INR.
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An elderly patient with Hx of Atrial Fibrillation on Warfarin was found to have INR of 7 He is on Warfarin. He has epistaxis (Minor bleeding). What should be done at the anticoagulant clinic?
INR 5-8 with or without minor bleeding) → STOP WARFARIN and Restart when INR is < 5. Epistaxis is a minor bleeding. (Note that if INR was > 8 ± minor bleeding, we would give vitamin K1)
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A patient presents with jaundice, dark urine and abdominal pain. After investigations → Heinz bodies and bite cells were found
. → G6PD
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patient has started taking anti-malarial medications after returning from abroad. He then develops mild jaundice, dark urine and tender right abdomen and palpable liver
. → Hemolytic Anemia (likely G6PD).
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A patient presents with Bloody Diarrhea followed by renal function tests impairment.
→ HUS (Hemolytic Uremic Syndrome
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A woman presents with irritability, Peripheral Paraesthesia, numbness, Impaired proprioception and low Hb.
→ Vitamin B12 Deficiency.
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10 YO boy presents with a low-grade fever and macular rash especially on the back of the legs following an upper respiratory tract infection. A few hours later, these macules have turned into purpuric lesions that do not blanch on glass test. The boy also complains of joint stiffness and headache. Hb (124 g/L) ▐ WBC (3.3 X 109/L) ▐ Platelets (219 X 109/L).
The Likely Dx → Henoch-Schonlein Purpura (HSP) Note that Hb, WBCs, and platelets are normal. Please note that if a patient presents with similar features but with LOW PLATELETS and normal Hb and WBCs, the answer would be → Idiopathic Thrombocytopenic Purpura
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Haemophilia (X-linked recessive, so the affected individual is a boy mainly) → ↑ PTT + (Bleeding into muscles or joints or easily bleeds).
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Henoch-Schonlein Purpura
HSP → PAAN: non-blanching Purpura ± Arthralgia, Abdominal pain, Nephropathy (Hematuria, Proteinuria) “not always” Purpura is non-blanching and mainly on the buttocks and Lower Limbs. • Precipitated by URTI – Sore Throat. • All Blood Results are NORMAL “Normal Hb, WBCs and Platelets”. • However, there might be ↑ ESR/ IgA/ Creatinine
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Purpura is non-blanching and mainly on the buttocks and Lower Limbs. • Precipitated by URTI – Sore Throat. • All Blood Results are NORMAL “Normal Hb, WBCs and Platelets”. • However, there might be ↑ ESR/ IgA/ Creatinine
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Idiopathic Thrombocytopenic Purpura (ITP)
Isolated Thrombocytopenia (low platelets) has to be given in a stem.
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man presents with night sweats, fever and lymphadenopathy.
→ Lymphoma.
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Manifestations of Lymphoma
√ Painless, Rubbery slowly progressive Peripheral lymphadenopathy. commonest”. √ Systemic manifestations → fever, malaise, fatigue, weight loss √ B Symptoms → Unintended Weight Loss “The (Late stage). ▐ Unexplained Fever ▐ Night Sweats. √ Splenomegaly, Hepatomegaly. √ BM is frequently involved → Pancytopenia → Anemia, Infections, Bleeding.
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A 3 YO child returned recently from India to the UK. He is lethargic and pale. His main diet is milk, with little to none of solid food. His lab results show low Hb, low MCV, low MCHC. What is the most likely diagnosis? A • Alpha thalassemia major. B • Beta thalassemia trait. C • Iron deficiency anemia. D • Folate deficiency. E • Vitamin B 12 deficiency.
The answer: → Iron deficiency anemia. “Milk decreases the absorption of iron” √ Alpha thalassemia major is usually fatal and causes hydrops fetalis. The baby would not survive gestation. √ Beta thalassemia trait is asymptomatic. It can result in lethargy and mild anemia in adulthood (not childhood or infancy). √ Folic acid and B12 deficiency cause Macrocytic anemia, however, the stem gives labs of microcytic anemia.
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A 40 YO man who has had bariatric surgery 3 years ago has been feeling lethargic in the past few months. His labs show Hb of 9, high MCV, normal iron studies. What is the likely diagnosis?
→ Vitamin B12 deficiency anemia • Since MCV is high → Macrocytic or megaloblastic anemia → Either vitamin B12 or folate deficiency. • Since the patient has had bariatric surgery (this could include gastric bypass or sleeve gastrectomy): Gastrectomy → Loss of the intrinsic factors in the stomach → impaired Vitamin B12 (Cobalamin) absorption → Vitamin B12 deficiency. • Rx → Intramuscular Hydroxocobalamin (vit b12) injections for life. Vitamin B 12 tablet are of no help as the problem is in the absorption (decreased absorption due to gastrectomy). Give it IM.
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A 19 YO ♂ known case of Sickle Cell Anemia presents with pallor, Shortness of breath, lethargy and headache. For the past week, he has been having flu- like symptoms. He also complains of joint aches on his hands, wrists, knees and ankles for the previous few days. His Hb is 56 g/L. Doctors suspected parvovirus B19 infection and sent specific IgM and IgG antibodies to Parvovirus B19.
Once you see parvovirus B19 in SCA or Hereditary Spherocytosis → Aplastic Crisis.
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Beta thalassemia AR
Beta Thalassemia Major (Autosomal Recessive) • Microcytic anemia. • Marked Pallor • Mild Jaundice • Has to receive Blood Transfusion frequently • Hepatosplenomegaly • Frontal Bone Bossing (Skull Bossing) • Frequent blood transfusions → Iron overload → Endocrinopathy e.g. DM. ◙ Rx: √ Lifelong Blood Transfusion (maintain Hb > 9.5). √ Iron Chelating Agents (e.g., Deferoxamine “Desferal” SC twice a week)
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And in adulthood if untreated, it can lead to frontal bossing and hepatosplenomegaly.
B thalassemia Minor (or thalassemia trait) is usually asymptomatic during childhood . However, in adulthood it may manifest as microcytic anemia with lethargy and mild anemia. • Remember, beta thalassemia Major starts since infancy and results in failure to thrive, vomiting feeds.
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Advantages of DOAC
♠ Give Warfarin or DOAC (Direct-Acting Oral AntiCoagulants, such as Apixaban, Rivaroxaban, Edoxaban, Dabigatran) To → ◙ All patients with score ≥ 2. ◙ Consider giving Warfarin or DOAC to Men whose scores ≥ 1. Advantages of DOAC: - No need for INR Monitoring, - Faster Onset of Action (2-4 hours), - Reduces the risk of intracranial Hemorrhage.
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Disadvantage of DOAC
Disadvantages of DOAC: - No Antidote - Require strict compliance by the patients. Note , it is important to remember DOAC Medications! They are asked in the exam in a stem which describes a patient with Atrial Fibrillation. We either give Warfarin or DOAC (e.g. Apixaban) as a long-term anticoagulant.
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After C-Section, a woman develops swelling of her entire left leg starting at the level just above the inguinal ligament. She also has Back and Buttock pain.
The likely cause → Common iliac VENOUS thrombosis. √ DVT is common in pregnancy and Post C-Section. ABOVE the level of inguinal BELOW the level of √ The Swelling in “iliac” VEIN thrombosis begins Ligament ± Back and Buttock pain. √ The Swelling in “Femoral” VEIN thrombosis will begin inguinal Ligament.
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Arterial occlusion
√ Arterial Occlusion does not present with Swelling like in this stem, it rather presents with the known ( 6P features of Acute Limb Ischemia) → Pain (Sudden), Pallor, Pulselessness, Paralysis, Paraesthesia (Numbness), Perishing cold.
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Dvt PE And dvla
Patients with DVT or Pulmonary embolism DO NOT NEED to inform DVLA and they can drive (no restrictions on driving for DVT or PE patients) as long as they haven’t experienced loss of consciousness and can move their legs.
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Chronic alcohol consumer + Gum bleeding, Petechiae
Think → Vitamin K deficiency. Especially if the issue is only for a few weeks. Chronic alcohol consuming can cause depletion of both vitamin K and C. However, vitamin K deficiency is a more common cause of bleeding in this case. Also, in vitamin K deficiency, serum PT would be low.
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VIT c def in alcoholics
Important: If there were additional things in the stem that describe scurvy “vitamin C deficiency” such as: Being an elderly, neglected and living alone “not eating fresh fruits” Pallor “anemia is a feature of vitamin C deficiency” Hypertrophic gums Then Pick → Vitamin C deficiency. Especially if the issue is for long eg, months. In short, vitamin K deficiency is a faster and a more common cause for bleeding in alcohol abusers. However, vitamin C deficiency can also occur in alcoholics especially of long time “as the total depletion of vitamin C storage takes more time than that of vitamin K depletion” and there will be other features besides bleeding that indicate vitamin C deficiency such as enlarged bleeding and friable gums, pallor, being elderly, neglected and malnourished.
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Important Points to Remember on Ischemic Stroke
• The antihypertensive of choice in patients with diabetes mellitus is → ACE inhibitors (eg, ramipril). • In patients with a history of ischemic stroke, the long-term management to prevent further stroke (2ry prevention of ischemic stroke) is as follows: √ Control Blood Pressure. Remember, if he has diabetes, pick or add ACEi eg, ramipril. √ Statins (for All patients regardless of their cholesterol baseline level). • If there is Atrial Fibrillation √ Ani-platelets (or) Anti-coagulation: (Based on presence or absence of AF): → Anticoagulants: Warfarin [or] DOAC (Dabigatran/ Apixaban/ Rivaroxaban/ Edoxaban). DOAC is now preferred. (Warfarin is almost obsolete nowadays. DOAC is recommended instead). • If No Atrial Fibrillation → Antiplatelets: Clopidogrel 75 mg OD. Important: What if a patient with a history of stroke is already on warfarin and presents with a new onset atrial fibrillation? Since he is already on an anticoagulant, he should continue on warfarin and should not be switched to DOAC (eg, Edoxaban) unless his INR is abnormal.
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A 77 YO man comes to the GP complaining of weight loss and fevers over the past 4 months. O/E, several cervical LNs are enlarged and painless. Blood results show: low Hb, high WBCs. LNs biopsy shows monomorphic small irregular B lymphocytes.
The likely Dx → Non-Hodgkin’s Lymphoma. • The given features are seen in lymphoma: Weight loss – loss of appetite – fever – Painless cervical lymphadenopathy • Monomorphic small irregular B lymphocytes can be seen in non-Hodgkin’s.
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Hodgkin and non Hodgkin differences
nucleated cells with prominent nucleoli), it is likely non-Hodgkin’s. This is to say: in Hodgkin’s lymphoma, there are Reed Sternberg cells (which are bi- or multinucleated cells with prominent nucleoli. If these cells are not seen, but other cells (eg, monomorphic, small, irregular B lymphocytes) and the symptoms go with lymphoma , then think of non-Hodgkin’s lymphoma. If the same scenario is given but without mentioning that LN biopsy has been done and asks you about the most appropriate (or diagnostic) investigation? → Lymph node biopsy. (important
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Megaloblastic anaemia differences Types
High MCV + Low Hb → Megaloblastic anemia Either vitamin B12 deficiency or Folate deficiency or Both. For Megaloblastic Anaemia (low Hb, high MCV): If the question asks about what is the best option test to specify the cause of this megaloblastic anemia, you have to choose either (Vitamin B12 assay) or (serum or red cell Folate). So, what to pick? √ If there are: Peripheral Paraesthesia “Pins and Needles sensation”, Loss of position and Vibration sense, Ataxia, confusion. → Vitamin B12 deficiency. √ If the patient does not eat vegetables and eat meat → Folate deficiency. √ If the patient is vegan (does not eat meat and dairy products) → Vitamin B12 deficiency. √ If the patient depends on microwavable food, canned food, with no vegetables → Folate deficiency. The key is to know that Vit.B12 is present in fish, meat, and dairy products while folic acid is present in vegetables. Note also that Folic acid stores in the body last for 4 months or less, while vitamin B12 store may last for up to 4 years!
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Patients on warfarin need to
→ Stop warfarin 5 days before surgery. • Patients on DOACs (eg, apixaban) need to → Stop warfarin 24 hours before surgery (if low bleeding risk surgery). → Stop warfarin 48 hours before surgery (if high bleeding risk surgery)
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Post-op patients who are expected to remain immobilized for a period of time need to be given:
→ Prophylactic dose of Low Molecular Weight Heparin e.g. enoxaparin. √ A prophylactic dose, not a treatment does. √ This is to help prevent DVT, Pulmonary embolism …etc
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Dvt mng
Important Update on DVT Management: Now, treatment doses of DOACs (e.g. Apixaban, Rivaroxaban) have become superior to Low Molecular Weight Heparin as anticoagulants. Thus, in the exam, if both treatment dose of apixaban/rivaroxaban and low molecular weight heparin are within the options , pick the one with rivaroxaban/apixaban.
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◙ This is to say, in suspected DVT (calf swelling, pain, pitting edema)
→ D-dimer, therapeutic dose of rivaroxaban, Leg Ultrasound in 24 hours.
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◙ What if the patient is already on the maximum dose of rivaroxaban (anticoagulant) and presents with calf pain, edema, swelling?
In this specific case, he is already on maximum dose anticoagulant, we request D-dimer and arrange leg U/S in 24 hours. After which, we may put him on different anticoagulants such as a combination of warfarin and LMWH.
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◙ What if these are 2 separate options (either D-dimer or U/S)?
→ Pick U/S as long as the DVT is likely.
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◙ What if DVT is unlikely?
◙ What if DVT is unlikely? → D-dimer may be enough.
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Patient with intracranial bleeding. INR is 4.6. He was given IV vitamin K. What is next to be given?
Intracranial Bleeding is a major bleeding. So, we should give both Vit K and PCC.
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A woman presents with lethargy, Raised MCV 108, Low Hb and tingling sensations over fingers. Best investigation?
◙ Remember the features of Vitamin B12 Deficiency: - Peripheral paraesthesia. - Impaired position and vibration (proprioception) sense. - Dementia → loss of memory, difficulties with thinking. - If untreated → permanent Ataxia.
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◙ A man on warfarin fell and presents with confusion. His INR was found to be 6.1 What should be done?
We suspect he has an intra-cranial hemorrhage (namely: subdural hematoma) Elderly + on warfarin + Hx of fall + presents with confusion/ headache Suspect → Subdural hematoma (In Neurology chapter). Since intracranial hemorrhage is a Major Bleeding, we give → Prothrombin complex concentrate.
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A man presents to the ER with epistaxis for the last 4 hours. Despite nasal packing and cautery, the nosebleed continues. His blood pressure is 77/41 mmHg. He takes warfarin for atrial fibrillation. His INR is 7 (target is 2-3). What is the most appropriate INITIAL step in managing this patient?
→ IV fluids. • Note that the patient is haemodynamically unstable (his SBP is < 90 mmHg) and the question asks about the INITIAL step! We need to stabilise him first with IV fluids or even blood transfusion in required. • Be careful to the word (initial management)!
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◙ A 63-year-old man with history of abdominal aortic aneurysm presents with sudden onset severe abdominal pain. He is on warfarin as he has a mechanical aortic valve replacement 6 years ago. Doctors suspect leaking abdominal aortic aneurysm. His blood pressure is 95/63 mmHg and HR is 120 bpm. What is the single most appropriate management?
Pcc
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Hemolytic Uremic Syndrome vs Thrombotic Thrombocytopenic
Triad: HUS 1) Hemolytic Anemia 2) Uremia (Acute Renal Failure) 3) Thrombocytopenia (Low Platelets) 4)Children Eating Undercooked Contaminated food → E. Coli O157 → Produce Verotoxin → Profuse Diarrhea → Bloody Diarrhea → (after 2-14 days) → Uremia “Acute Renal Failure” (Hematuria, Proteinuria, ↑ Urea and Creatinine) So, remember: • Diarrhea → turns Bloody → Renal Failure (Acute Kidney Injury) (Hematuria, proteinuria…etc). ± Features of Anemia (e.g. Pallor, Fatigue). Rx → Supportive √ IV fluids. √ ± Blood Transfusion √ ± Dialysis (if required) √ If Very Severe → Plasma Exchange ◙ Never Give Antibiotics in HUS! (More toxins are released as the E. Coli dies)
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TTP
Pentad: TTP 1) Hemolytic Anemia 2) Uremia (Acute Renal Failure) 3) Thrombocytopenia (Low Platelets) 4) Neurological Manifestations 5) Fever ADAMTS 13 factor Deficiency or inhibition
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5-year-old girl with 5 days history of bloody diarrhoea and dehydration. Blood culture shows E. coli. Lab shows that: Serum Na+ is low, serum K+ is high, serum Urea and Creatinine are high, Serum Calcium is Normal, Serum Bicarbonate is low. What is the like diagnosis?
This is a classic case of HUS (Hemolytic-Uremic Syndrome). √ HUS is the most common cause of acute kidney injury in children and is increasingly recognized in adults. AKI in HUS
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Example (3), A 44-year-old woman presents to the ER with a diagnosis of hemolytic uremic syndrome. Over the last week, she has been experiencing fatigue, dizziness, and shortness of breath. Lab and examinations reveal pallor, tachycardia, hypotension, low serum hemoglobin (60 g/L), moderately deteriorated renal function tests. What is the most appropriate initial management?
Immediate administration of packed red blood cells transfusion. Remember: the management of HUS “hemolytic uremic syndrome” is supportive (eg, blood transfusion, dialysis, IV fluids). • This patients’ symptoms (fatigue, dizziness, tachycardia, pallor, shortness of breath) are caused by the severe anemia. Thus, RBCs transfusion is more appropriate than dialysis initially in this case. • Remember: in HUS, do not give antibiotic
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66 YO ♂ presents with Hoarseness of voice. He has a Hx of weight loss recently. Chest X ray shows opacity in the right upper mediastinum. There is no Shortness of breath. What is the single best Investigation to diagnose?
Answer → Lymph Node Biopsy This is a tricky question. ◙ Opacity/ mass in the superior mediastinum could be one of the (5 Ts) √ Terrible Lymphoma. √ Thymus (Thymoma). √ Thoracic Aortic Aneurysm √ Thyroid Goitre/ Neoplasm. √ Teratoma. ◙ Looking at the History, No SOB or Cough → R/O Lung carcinoma. ◙ There is a Hx of Weight Loss + Old Age → The best one of the 5 Ts that fits well here is Lymphoma. ◙ To Diagnose Lymphoma, Lymph Node Biopsy is required. ◙ Note, Lymphoma can compress a Unilateral Recurrent Laryngeal Nerve leading to Hoarseness of voice.
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Pancoast tumour Left and right supra clavicular nodes
◙ Additional Notes to help you correlate topics: • Left Supraclavicular Mass → Virchow’s Node → Indicative of Gastric Carcinoma (Anorexia, Dyspepsia, Weight Loss, Old age). This sign is called → Troisier’s sign. • Right Supraclavicular Mass → Oesophageal cancer, Lung cancer, Hodgkin’s Lymphoma. • Pancoast Tumour → A tumour of the Apex of the Lung (located at the top end of either the left or the right lung ). It typically spreads to the nearby tissues such as the Ribs and the Vertebrae. Most Pancoast tumours are Non-small cell lung cancer.
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Macrocytic Anemia (Highmcv) plus AI condition
Vitiligo, Hypothyroidism, DM type 1, Psoriasis Think of Pernicious Anemia. Pernicious Anemia → Autoimmune gastric atrophy → Loss of gastric intrinsic factors → impaired Vit. B12 absorption → Vit. B12 deficiency. ◙ Management in such a case would be → Intramuscular Hydroxocobalamin -Vit B12- (not Oral, nor IV). Important √
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44 YO ♀ presents with tiredness and fatigue. Her Hb is 88 g/L (Low), MCV is 125 (High). Vitiligo is noted on her hands.
The likely Dx → Pernicious Anemia. Another Valid → Vit. B12 deficiency. √ Pernicious Anemia is an Autoimmune disease. It causes Vit. B 12 deficiency √ Given the elevated MCV and her autoimmune history ( eg, vitiligo, hypothyroidism, DM-1), vitamin B12 deficiency is the most likely cause of her anaemia, likely due to pernicious anaemia. This condition is common in individuals with other autoimmune diseases, where the body's immune system attacks cells in the stomach, reducing intrinsic factor production, which is essential for vitamin B12 absorption. The reduced B12 levels impair red blood cell production, leading to macrocytic anaemia. √ While folate deficiency can also cause macrocytic anaemia, in patients with autoimmune backgrounds, B12 deficiency is more likely. In clinical practice, B12 levels would be measured to confirm the diagnosis. (High MCV = Macrocytic Anemia). Presence of another Autoimmune disease (such as Vitiligo, Hypothyroidism) supports the Dx of Pernicious Anemia.
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A 44 YO ♀ presents with tiredness and fatigue. Her Hb is 88 g/L (Low), MCV is 125 (High). She also complains of constipation, coarse dry skin, hair loss and cold peripheries.
She has Hypothyroidism (which is mostly an Autoimmune disease - Hashimoto thyroiditis). Macrocytic Anemia + Associated Autoimmune disease (such as Vitiligo/ Hypothyroidism) →  Pernicious Anemia. Another → Vitamin B12 deficien
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