Case 11- Anaemia Flashcards by pio monaghan

General management of sickle cell anaemia

Avoid dehydration/ other triggers
Ensure vaccines up to date (flu and pneumococcal)
Antibiotic prophylaxis
Hydroxycarbamide- stimulates production of foetal Hb
Blood transfusion if severe anaemia
Exchange transfusion during acute crises
Bone marrow transplant can be curative

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Signs and symptoms of anaemia

Fatigue, dyspnoea, faintness, palpitations, headache, tinnitus, angina, conjunctival pallor, SOB on exertion, atrophic glossitis, angular stomatitis, koilonychia, hair loss

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Use osce stop when doing anaemia

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Anaemia of chronic disease vs iron deficiency anaemia

Hb- low in both

Ferritin: low in iron deficiency , normal/high in ACD

Serum Iron: low in both

Transferrin saturation: low in both

Total iron binding capacity- high IDA, low AOCD

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Blood film findings

Sickle cells
Schistocytes- microangiopathic haemolytic anaemia (HUS/ TTP)
Inclusion bodies- malaria
Spherocytes/ elliotocytes- hereditary spherocytosis/ elliptocytosis
Heinz bodies/bite and blister- G6PD deficiency
Prickle cells- autoimmune haemolytic anaemia
Acanthocytes- splenectomy/ ALD
Blast cells- leukaemia, haematological malignancy
Howell jolly bodies- post splenectomy and hyposplenism: sickle cell, coeliac disease, IBD
Tera drop poikilocytes- Myelofibrosis
Reticulocytes- haemolysis, haemorrhage
Target cells- have a ring of pallor. Liver disease, hyposplenism, thalassaemia

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Aplastic anaemia

Pancytopenia and hypoplastic bone marrow
Peak incidence- 30 years

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Causes of aplastic anaemia

Idiopathic
Congenital eg. Fanconi anaemia
Drugs- cytotoxic, chloramphenicol, sulphonamides, phenytoin, gold, benzene
Infection
Radiation

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Autoimmune haemolytic anaemia

Divided into warm or cold depending on what temperature the antibodies cause haemolysis
Mostly idiopathic but can be secondary to infection, drugs, lymphoproliferative disorder
Characterised by a positive Coombs test (direct antiglobulin)

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Warm autoimmune haemolytic anaemia (AHA)

IgG
Occurs in the spleen
Causes
-SLE
-neoplasia eg. Lymphoma, CLL
-methyldopa

Steroids, immunosuppression, splenectomy

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Cold AIHA

IgM
Complement
Raynauds and acrocyanosis

Causes
-neoplasia (lymphoma)
-infection (mycoplasma, EBV)

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Fanconi anaemia

Haem- aplastic anaemia, AML
Neuro abnormalities
MSK- short stature, radial abnormalities
Cafe au lair spots

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Features of GPD6 deficiency

Haemolytic anaemia- neonatal jaundice
Gallstones
Splenomegaly
Heinz bodies and bite and blister cells seen on blood film

NB- difference with hereditary spherocytosis is the cells seen on a blood film

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Diagnosing GPD6 deficiency

GPD6 enzyme assay 3 months after an acute episode of haemolysis

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GPD6 haemolytic crisis precipitants

Infection
Beans
Drugs- anti malarials eg. Primaquine, cirpofloxacin, sulph drugs eg. Sulphonamides, sulfonylureas (glicazide), sulphasalazine

NB- Heinz bodies

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Iron deficiency Anaemia investigations

FBC
Iron studies (serum ferritin low (but it’s an inflammatory protein so may be raised), TIBC high, transferrin saturation low)
Blood film- target cells, pencil poikilocytes, anisopoikilocytosis (red blood cells of different shapes and sizes)
Endoscopy- males and post menopausal females who present with iron deficiency anaemia (2 week wait for endoscopy)- urgent

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Management of iron deficiency anaemia

Manage underlying cause
Iron rich diet- dark green leafy vegetables, meat, iron fortified bread
Oral ferrous sulphate- keep taking for 3 months after it has been corrected. SE’s include nausea, abdominal pain, constipation, diarrhoea, black stool

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Paroxysmal nocturnal haemaglobinuria

Acquired disorder leading to haemolysis of any cells

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Features and diagnosis of PNH

Haemolytic anaemia
Pancytopenia
Haemaglobinuria esp. in morning
Thrombosis eg. Budd chiari syndrome
Aplastic anaemia

Flow cytometry (CD59/ CD55)

Management of PNH

Blood product replacement
Anticoagulation
Stem cell transplantation

Causes of sideroblastic anaemia

Myelodysplasia
Alcohol
Lead
Anti TB medications

Investigations for sideroblastic anaemia

FBC- microcytic anaemia
Iron studies- high ferritin, iron, transferrin saturation
Blood film- basophilic stippling of RBC’s
Bone marrow- Prussian blue staining will show ringed sideroblasts

Sickle cell disease

Autosomal recessive haemolytic anaemia which predisposes to vasculo-occlusive crises

Features of sickle cell disease

General- dactylitis, chronic haemolytic anameia (fatigue, pallor, weakness)

Vasculo-occlusive crises- bone, abdominal or chest pain. Ulcers and a vascular necrosis. Triggered by cold, dehydration, hypoxia, or infection.

Aplastic crisis- parvovirus B19 (pancytopenia)

Sequestration crises- pooling of blood in liver, spleen causing organomegaly (but spleen may also be atrophied due to repeated splenic infarcts)

Treatment of a sickle cell crisis

analgesia e.g. opiates
rehydrate
oxygen
consider antibiotics if evidence of infection

blood transfusion
-indications include: severe or symptomatic anaemia, pregnancy, pre-operative
-does not rapidly reduce the percentage of Hb S containing cells

exchange transfusion
-indications include: acute vaso-occlusive crisis (stroke, acute chest syndrome, multiorgan failure, splenic sequestration crisis
-rapidly reduces the percentage of Hb S containing cells

Investigations for sickle cell anaemia

Usually picked up with the newborn heel prick test FBC- anaemia, LFT, UE, blood film (sickle shaped RBC’s, Howell jolly bodies) Haemaglobin electrophoresis (definitive diagnosis)

Features of Thalassaemia

Haemolytic anameia (RBC’s are fragile and breakdown easily) Microcytic anaemia, fatigue, pallor, jaundice, gallstones, Splenomegaly, poor growth and development, pronounced forehead and malar eminences

Investigations for Thalassaemia

FBC (microcytic anaemia), blood film Haemoglobin electrophoresis

Alpha Thalassaemia

Blood transfusions Splenectomy Bone marrow transplant may be curative

Beta Thalassaemia major

Failure to thrive Regular transfusions Splenectomy Bone marrow transplant may be curative

Iron overload in Thalassaemia

As a result of faulty RBC’s, recurrent transfusions, increased iron absorption in response to the anameia Patients have serum ferritin checked regularly Management involves limiting transfusions and iron chelation

Features of iron overload

Like haemachromatosis; Fatigue, cirrhosis, infertility, impotence, heart failure, arthritis, diabetes, osteoporosis, joint pain Desferrioxamine may help

B12 deficiency

Anaemia symptoms eg. Breathlessness Peripheral neuropathy with numbness or Parasthesia Mood or cognitive changes Visual changes Loss of vibration sense or proprioception Subacute spinal cord degeneration

Investigations for B12 deficiency

FBC, blood film Serum B12 Intrinsic factor antibody (gold standard)

Investigating haemolytic anaemia

Urinalysis- haemaglobinuria FBC, UE, LFT, serum bilirubin, LDH, blood film Coombs test (autoimmune) Hb electrophoresis Osmotic fragility testing Enzyme assays

What is hereditary spherocytosis

Most commonly hereditary haemolytic anaemia in people on Northern European descent Autosomal dominant Sphere shaped RBC Spleen destroys RBC’s- get Splenomegaly over time

Presentation of HS

Failure to thrive, jaundice, gallstones Splenomegaly, aplastic crisis precipitated by parvovirus infection Degree of haemolysis variable

Diagnosis and Management of HS

Acute haemolytic crisis- supportive, transfusion Long term treatment- folate replacement, splenectomy Diagnosis- EMA binding, cryohaemolysis test, electrolysis may also be used

G6PD deficiency vs HS

Male vs male/female African and Mediterranean vs Northern European Intravascular vs extra vascular haemolysis (hence splenomegaly and possible splenectomy) Heinz bodies in G6PD

High reticulocyte count in sickle cell anaemia

Possible due to haemolytic crisis or acute sequestration NB- low reticulocytes- think parvovirus

Sequestration crisis

sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia associated with an increased reticulocyte count

Aplastic crisis

caused by infection with parvovirus sudden fall in haemoglobin bone marrow suppression causes a reduced reticulocyte count

Blood film for hyposplenism/splenectomy

Acanthocytes (S/H) Howell jolly (S/H) Target cells (H)

Beta thalassaemia trait

Beta-thalassaemia trait is an autosomal recessive condition characterised by a mild hypochromic, microcytic anaemia. It is usually asymptomatic Features mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia HbA2 raised (> 3.5%)