Haematology Flashcards
Summarise Iron Deficiency Anaemia
Causes: diet, pregnancy, bleeding, poor absorption of iron in duodenum/ jejunum
Sx: normal anaemia signs (fatigue, SOB, palpitations, dizzy, pale, subconjunctival pallor, tachycardia), pica, hair loss/ brittle hair, koilonychia (spoon shaped nails), angular cheilitis, atrophic glossitis
Ix: Transferrin (protein that binds to iron); TIBC (proportion of transferrin bound to Fe) - both increase in fe deficiency, ferritin (how iron is stored- high ferritin usually indicates inflammation). Pencil cells + target cells seen on blood film.
Mx: investigate new Fe deficiency without clear underlying cause via OGD and colonoscopy. PO ferrous sulfate 200mg TDS
Summarise Pernicious Anaemia
Pathophysiology: Autoimmune condition where antibodies form against parietal cells which produce intrinsic factor which is needed to absorb B12. B12 deficiency can also be caused by insufficiency in diet
Sx: B12 deficiency → neuro issues eg peripheral neuropathy, loss of proprioception/ vibration, visual changes, cognitive and mood changes
Ix: Auto-antibodies (intrinsic factor Ab, Gastric parietal cell Ab), B12, folate, hypersegmented neutrophils on blood film
Mx: If there is a lack in diet can be PO replacement but if severe/ pernicious replace with IM hydroxocobalamin TDS twice a week. Treat B12 deficiency BEFORE folate replacement as otherwise there is a risk of subacute combined degeneration of the cord
Summarise Haemolytic Anaemias
Pathophysiology: RBC breakdown e.g. spherocystosis, elliptocysosis, thalassemia, SCD, G6PD def.
Can also occur small blood vessels secondary to: HUS, DIC, TTP, SLE, cancer
Presentation: anaemia, Jaundice, splenomegaly (spleen filled with destroyed RBC), aplastic crisis in parvovirus infection
Spherocytosis: most common, AD, give folates and spleenectomy
G6PD Deficiency: X linked recessive, Fava beans/ abx/infections trigger, heinz bodies on blood film
Ix: normocytic anaemia, blood film shows schistocytes, DCT positive, reticulocytes raised.
Summarise Thalaseemia
Pathophysiology: Defect alpha and beta chains, AR inheritance
Presentation: fatigue, pallor, jaundice, fallstone, splenomegaly, FTT, prononced forehead and malar eminences (Bone marrow expansion –> more RBC)
Ix: microcytic anaemia (as missing a chain), Hb electrophoresis
Mx: Transfuse, careful of iron overload (chelation), splenectomy, bone marrow transplant
Summarise Sickle Cell Anaemia
Pathophysiology: AR causing RBC crescent shaped –> polymerise in cold
Presentation: Afro-carribean. Anaemia, Sickle cell crisis, stroke, avascular necrosis, priapism, infection, CKD, acute chest syndrome, splenic sequestation crisis, aplastic crisis, gallstones, visual problems, ulcers
Ix: Newborn screening
Mx: Hydrate, vaccines, Pen V for prophylaxis, hydroxycarbamide to produce HbF, bone marrow transplant. Crizanlizumab if regular pain.
Crisis: Admit, keep warm, fluids, analgesia
Summarise Leukaemia
Pathophysiology: Bone marrow cancer which leads to anaemia, leukopenia and thrombocytopenia
Types:
To remmber age of presentation: ALL CeLLmates have CoMmon AMbitions
CLL → CML –> AML (most common to least)
ALL: Children, high lymphocytes, Down’s syndrome associated.
CLL: Smear/ smudge cells, high lymphocytes, high WCC, band cells, most common overall, may tranform into lymphoma
CML: associated with philadelphia chromosome, high WCC, band cells, raised platelets, normal neutrophils, 3 phases, massive spleenomegaly, treat with imatinib
AML: Linked to myeloproliferative disorders e.g. polycythema ruby vera, auer rods, neutropenia, blast cells, often thrombocytopenia
General Presentation: fatigue, bruising, infections, fever, pallor, bleeding abnormally, hepatosplenomegaly
Ix: FBC, blood film, lactate dehydrogenase, bone marrow biopsy = definitive, CXR for mediastinal lymphadenopathy, lymph node biopsy.
Mx: chemo, radio, blood marrow transplant, steroids
Summarise Lymphoma
Non-Hodgkin’s vs Hodgkins: H has reed-sternberg cells on biopsy of lymph nodes
RF: EBV, autoimmune conditions, HIV
Sx: lymphadenopathy (non tender, rubbery, pain with alcohol), fatigue, fever, night sweats
Ix: biopsy node, lactate dehydrogenase, imaging
Ann Arbor staging used - using diaphragm to distinguish stages
Mx: chemo, radio, monoclonal antibodies, stem cell transplant
Summarise Myeloma
Cancer of plasma cells (make Ab/Ig). One of the Ig multiple massively → IgG most common
Causes leukoaemia sx because of bone marrow infiltration; there is bony disease with increased clast activity which causes osteolytic lesions eg raindrop skull, #s and hypercalcaemia
MGUS precursor found via bence jones protein in urine (light chain of the Ab). The Ig can also impair renal function by blocking up + hypercalcaemia impairs kidney
Presents with CRAB: hypercalcaemia, renal failure, anaemia, bone lesions/ pain
Ix: if suspect do initially: FBC, calcium, ESR, plasma viscosity (raised by Ig) then
→ serum protein electrophoresis + Ig + light chain assay,
urine electrophoresis for urine bence jones,
bone marrow biopsy,
MRI -> CT -> skeletal survey
Mx: MDT, chemo, stem cell transplant, VTE prophylaxis, bisphosphonates, ortho surgery
Summarise thrombocytopenia
Low platelets which can be due to: sepsis; B12 def; liver failure; leukaemia; myeloma; meds; alcohol; ITP; TTP; HUS
Present: easy bleeding eg nosebleeds, bleeding gums, heavy periods, bruising, blood in urine/ stool
Idiopathic thrombocytopenia: usually in children, have purpuric rash, autoimmune issue where Ab are created against platelets → mx with steroids, IV Ig, monoclonal Ab, splenectomy, avoid contact sports + blood thinners
Thrombotic thrombocytopenic purpura: tiny blood clots in small vessels use up platelets due to problems with protein that switch off vWF. Mx is with steroids, plasma exchange, monoclonal Ab
Summarise Haemophilia
X Linked recessive. Haemophilia A effects factor 8 and haemophilia B (Christmas disease) effects factor 9
Present: bleeding (like thrombocytopenia), spontaneous bleeding into joints and muscles
Mx: IV infusion of missing clotting factors, desmopressin to release vWF, tranexamic acid (antifibrinolytic)
Summarise Von Willebrand disease
AD deficiency of vWF.
Sx: bleeding easily, heavy and prolonged
Mx: desmopressin, vWF infusion, factor 8