Haem investigations and treatment Flashcards

1
Q

Iron deficiency anaemia

Investigations
Treatments

A

Investigations
1st - Iron studies –> Decreased serum Fe, decreased ferritin, decreased transferrin saturation, increased TIBC

TIBC - total iron binding capacity - space for iron to attach on transferrin molecules
- Transferrin saturation - proportion of transferrin molecules bound to iron.

–> Peripheral blood smear (microcytic, hypochromic RBC)

Treatment
1st - Oral iron replacement - ferrous sulphate. If FS poorly tolerated then ferrous gluconate

IV iron replacement

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2
Q

Thalassemia

Investigations
Treatments

A

Investigations
1st - FBC + peripheral blood smear (microcytic anaemia, hypochromic RBC, increased reticulocyte, tear drops (BM not producing normal RBC)

GS - Haemoglobin electrophoresis (diagnose globin abnormalities)

X-ray of skull - hair on end appearance (increased BM activity)

Treatment
Alpha - Folate supplements + blood transfusion

Beta
1st - Regular blood transfusion + iron monitoring + iron chelation therapy (desferrioxamine)

Splenectomy - if massively enlarged and at risk of rupture
Folate supplements (for haemolytic anaemia)
Definitive - BM stem cell transplant

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3
Q

Sickle Cell Anaemia

Investigations
Treatment

A

INVESTIGATIONS
(For newborns - heel prick test)
1st FBC + peripheral blood smear - Normocytic, normochromic with increased reticulocytes. + sickled RBC and Howell Jolly bodies (RBC with DNA indicate hyposplenism)

GS - Hb electrophoresis - diagnostic with proportion of HbS 75-90%

TREATMENT
- For acute complications e.g. Acute chest crisis - analgesia + oxygen.
- If infection e.g. parvovirus B19 - aplastic anaemia give antibiotics

LONG TERM
- HYDROXICARBAMIDE (increases HbF)
- Blood transfusion + iron chelation

Last resort - BM stem cell transplant

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4
Q

G6PD deficiency

Investigations
Treatments

A

INVESTIGATIONS
1st line - FBC + blood smear/film - Normocytic, normochromic RBC, increased reticulocytes. + HEINZ bodies (indicate oxidative injury to erythrocytes - denatured haemoglobin)

GS - decreased serum G6PD levels

TREATMENT
1st - Avoid precipitants e.g. Fava beans and certain medication (nitrofurantoin, trimethoprim)

Blood transfusion if rapid anaemia and jaundice

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5
Q

Autoimmune haemolytic anaemia

Investigations
Treatments

A

INVESTIGATIONS
- Direct antigloublin test (COOMBS) - positive suggests antibody bound to patient’s own cells. (Negative - hereditary spherocytosis - spectrin membrane protein deficiency)

Bite cells?

TREATMENT
- Blood transfusion (iron chelation therapy)
- Prednisolone - corticosteroid that reduces antibody production
- Rituximab - targets CD20 on B lymphocytes (decreasing antibody production)

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6
Q

Folate deficiency anaemia

Investigations
Treatments

A

INVESTIGATIONS
FBC + blood smear - Macrocytic, megaloblasts

Serum Folate - low
(Possible concomitant B12 deficiency)

TREATMENT
1st - Dietary advice then oral folic acid replacement

If concomitant B12 deficiency - replace B12 first (can cause subacute combined degeneration of the spinal cord)

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7
Q

Pernicious anaemia

Investigations
Treatments

A

INVESTIGATIONS
1st line - FBC and blood film –> Macrocytic anaemia + megaloblasts (hypersegemented nucleated neutrophils with more than 6 lobes)

  • Decreased Serum B12

GS - Anti IF and anti parietal antibodies

Treatment
Dietary advice - Salmon, Eggs

1st line - B12 supplements –> IM Hydroxocobalamin

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8
Q

Acute myeloid leukaemia

Investigations
Treatments

A

INVESTIGATIONS
FBC + blood film - Pancytopenia, presence of myeloid blasts and AUER RODS (+ positive myeloperoxidase stain)

Diagnostic –> BM biopsy and aspirate - >10% infiltration by myeloid blasts

TREATMENT
Chemotherapy + All trans retinoic acid (vitamin A that helps with growth and development of cells)
(Allopurinol may be given to prevent tumour lysis syndrome)

Consider: Antibiotic prophylaxis for neutropenia/transfusions for anaemia

Last resort: BM transplant

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9
Q

Chronic myeloid leukaemia

Investigations
Treatments

A

INVESTIGATIONS
First line - Anaemia, thrombocytopenia but elevated granulocytes

DIAGNOSTIC - Philadelphia chromosome genetic test (9:22)

BM biopsy - elevated granulocytes

TREATMENT
Chemotherapy + IMATINIB (tyrosine kinase inhibitor)

(Possible allopurinol for TLS)

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10
Q

Chronic lymphoid leukaemia

Investigations
Treatments

A

Investigations
1st FBC - Elevated WBC count (Anaemia, thrombocytopenia)
Blood film – smudge cells present (fragile lymphocytes)

Immunoglobulin levels - low, hypogammaglobulinemia (ordered for patients with recurrent infections)

Treatment
1st - Chemotherapy (with allopurinol)
IV immunoglobulin for treating hypogammaglobulinemia

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11
Q

Acute lymphoid leukaemia

Investigations
Treatments

A

INVESTIGATIONS
1st FBC - Pancytopenia
Blood film - increased lymphoblasts
GS - BM biopsy and aspiration > 20% lymphoblasts

TREATMENT
Chemotherapy (possible allopurinol)

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12
Q

Hodgkin’s lymphoma

Investigations
Treatments
Side effect of treatment

A

INVESTIGATIONS
GS - Excisional lymph node biopsy –> Reed sternberg positive (b lymphocytes with 2 nuclei and a prominent nucleoli)

  • ESR - elevated
  • FBC - decreased haemoglobin
    Imaging CT scan of lymph nodes (evaluate extent of disease) and stage via ANN ARBOUR system

TREATMENT
ABVD chemotherapy (combination) - Adriamycin, bleomycin, vimblastine, doxarbazine

(Interim CT scan to assess metabolic response)

Side effects: Alopecia, n+v, infection, FEBRILE NEUTROPENIA (fever, tachycardia, sweats, tachypnoea) –> treated with amoxicillin + fluoroquinolone

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13
Q

Brief way to differentiate between the 4 types of leukaemia

A

AML - Associated with Auer rods (+ downs and patau’s syndrome)

CML - Associated with the Philadelphia chromosome

CLL - Associated with smudge cells, hypogammaglobulinaemia (richter transformation complication)

ALL - Most common leukaemia in children, associated with Downs

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14
Q

Non Hodgkin’s lymphoma

Investigations
Treatment

A

Investigations
GS - excisional lymph node biopsy –> no reed sternberg cells
(A starry sky subtype would confirm Burkitt’s lymphoma)

(Fluorodeoxyglucose) CT scan for staging via Ann Arbour

TREATMENT
R-chop (21 days x 3) - combination chemotherapy
- Rituximab, cyclophosphamide, doxorubicin, oncovin (vincristine) and prednisolone.

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15
Q

Multiple myeloma

Investigations
Treatment

A

INVESTIGATIONS
FBC - anaemia
Blood smear - rouleax formation
Serum electrophoresis - M spike (M protein is abnormal - MGUS)
Urine electrophoresis - Bence Jones Proteins
Serum calcium - increased
Serum creatinine - increased due to renal impairment

GS - Bone marrow biopsy - >10% infiltration by monoclonal plasma cells

Treatment
Chemotherapy (dexamethasone)
Bisphosphonates (zoledronic acid)

Consider: Stem cell transplant

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16
Q

Polycythaemia + Polycythaemia Vera (treatment)

Investigations
Treatment

A

INVESTIGATIONS
FBC - increased RBC (possible increased WBC and platelets also)
- Haematocrit (percentage by volume of RBC in the blood) - >49% for men and >48% for females
- Haemoglobin >165 in men, >160 in women
- Genetic test - JAK2V617F mutation present

TREATMENT
- Venesection + aspirin (reduce risk of thrombosis in patients)

If patient is at high risk of thrombosis (has had history of venous thromboembolism event) - Consider Chemotherapy with HYDROXICARBAMIDE

17
Q

Haemophilia A and B

Investigations
Treatment

A

INVESTIGATIONS
- Prolonged APTT (+ normal PT)
- Coagulation factors 8 and 9 assay - showing decreased or absent factor levels

TREATMENT
Haemophilia A - IV factor 8 (desmopressin)
Haemophilia B - IV factor 9

18
Q

Von willebrand disease

Investigations
Treatment

A

INVESTIGATIONS
- Normal PT and prolonged APTT
- Normal Factor 8 and 9 assay (to eliminate haemophilia)
- Decreased VWF antigen (diagnostic) <0.3

TREATMENT
- Desmopressin (stimulates VWF release from endothelial cells)
- Von willebrand factor infusion

(Tranexamic acid)

19
Q

Disseminated intravascular coagulation

Investigations
Treatment

A

Investigations
- FBC - low platelet count
- Fibrinogen decreased
- Elevated D dimer (fibrin degradation product)

Schistocytes can be seen on blood film

TREATMENT
- Treat underlying disorder (e.g. trauma, sepsis + protect organs) + FRESH FROZEN PLASMA for replacement of coagulation factors + platelet transfusion

20
Q
A