Haem investigations and treatment Flashcards
(20 cards)
Iron deficiency anaemia
Investigations
Treatments
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
Thalassemia
Investigations
Treatments
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
Sickle Cell Anaemia
Investigations
Treatment
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
G6PD deficiency
Investigations
Treatments
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
Autoimmune haemolytic anaemia
Investigations
Treatments
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)
Folate deficiency anaemia
Investigations
Treatments
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)
Pernicious anaemia
Investigations
Treatments
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
Acute myeloid leukaemia
Investigations
Treatments
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
Chronic myeloid leukaemia
Investigations
Treatments
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)
Chronic lymphoid leukaemia
Investigations
Treatments
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
Acute lymphoid leukaemia
Investigations
Treatments
INVESTIGATIONS
1st FBC - Pancytopenia
Blood film - increased lymphoblasts
GS - BM biopsy and aspiration > 20% lymphoblasts
TREATMENT
Chemotherapy (possible allopurinol)
Hodgkin’s lymphoma
Investigations
Treatments
Side effect of treatment
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
Brief way to differentiate between the 4 types of leukaemia
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
Non Hodgkin’s lymphoma
Investigations
Treatment
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.
Multiple myeloma
Investigations
Treatment
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
Polycythaemia + Polycythaemia Vera (treatment)
Investigations
Treatment
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
Haemophilia A and B
Investigations
Treatment
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
Von willebrand disease
Investigations
Treatment
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)
Disseminated intravascular coagulation
Investigations
Treatment
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
