Haematology revision

Shin Ying Lee

2010

• You are on cover for a surgical ward when you are called to Mrs McCulloch, who has just commenced a blood transfusion for a low post operative haemoglobin. Three minutes after the start of the transfusion the patient develops lumbar pains, rigors, dyspnoea and hypotension.

2010

• You are on cover for a surgical ward when you are called to Mrs McCulloch, who has just commenced a blood transfusion for a low post operative haemoglobin. Three minutes after the start of the transfusion the patient develops lumbar pains, rigors, dyspnoea and hypotension.

• What is the name of this complication of the transfusion. 1

Acute hemolytic transfusion reaction

• What is the most likely procedural reason for this complication to have arisen? 1

ABO incompatibility. Failure to check identity of patient when taking sample for compatibility testing, failure to perform paper identity checks before blood is transfused.

• List 3 treatments that the patient might then urgently require. 3

1.High flow oxygen2.IV fluids3.Diuretics

• Name 2 further steps that would need to be taken rapidly. 1

1. Send donor blood back to blood bank with notification of event

2. Inform hospital transfusion department immediately

• What might you detect in the urine? 1

Hemoglobin

• List three further short term complications of blood transfusions. 3

1. Febrile non-hemolytic reaction 2. Allergic and anaphylactic reaction 3. TRALI (Transfusion related acute lung

injury)

2007

• A 40 year old woman presents with recent tiredness and lethargy. She has pale conjunctivae and you think there may be some yellowing of the sclera.

2007

• A 40 year old woman presents with recent tiredness and lethargy. She has pale conjunctivae and you think there may be some yellowing of the sclera.

• Name two investigations you would perform and what are you likely to see? 2

1. Bloods – FBC, LFT. high reticulocytes count, High bilirubin levels>50

2. Peripheral blood film- spherocytes, elliptocytes

2. Urine sample – Urobilinogen in the urine

• What two abnormalities might you expect to see on biochemistry and haematology in haemolytic anaemia? 2

Biochem- High bilirubin levels, high LDH Haemato- Low hemoglobin, increase

reticulocytes count

• She has a splenectomy. What two things would you want to vaccinate against? 2

Pneumococcal vaccine Haemophilus influenza type B vaccine

• What two pieces of advice would you want to give her in regards to her asplenism?2

1) Lifelong prophylactic antibiotics 2) Reimmunisation every 5 years, annual

influenza vaccine 3) Increased risk of malaria falciparum, anti-

malarial precautions

• Name two causes of haemolytic anaemia. 2

1) Abnormal membrane (Hereditary spherocytosis, elliptocytosis)

2) Abnormal enzymes (G6PD deficiency) 3) Abnormal hemoglobin synthesis

(thalassemia, hemoglobinopathies, sickle cell disease)

Leukaemia

• 4 types Acute myeloid leukaemia (AML) Acute lymphoid leukaemia (ALL) Chronic myeloid leukaemia (CML) Chronic lymphocytic leukaemia (CLL)

Investigations

• Blood count, White count differentials, red cells, haemoglobin, plaletets

• Peripheral blood film• Bone marrow biopsy and aspiration• Immunophenotyping and molecular methods• Cytogenetic analysis

CML

• Anaemia, bruising, unwell• Dragging sensation at the abdomen/ abdomen

fullness (splenomegaly)• Translocation of 9: 22. Philadelphia chromosome

>80% cases • Chimeric gene (BCR/ABL) > phosphoprotein

(p190 and p210) with high tyrosine kinase activity seen.

• spectrum of myeloid lineage cells

CML(peripheral blood film)

• Three phrases - Chronic phase - Accelerated phase - Blast crisis

Treatment

• Imatinib (tyrosine kinase inhibitor) Glivec/Gleevec

• Allogenic transplantation

CLL

• Swollen glands• Constitutional signs and symptoms: Fever,

night sweats, weight loss• Mature lymphocytes• Transformed to Diffuse Large B cell lymphoma Richter’s transformation• Chemotherapy • Stem cell transplant

CLL (peripheral blood film)

AML

• Neoplastic proliferation of blast cells derived from myeloid elements.

• Bone marrow aspiration: auer rods, myeloid precursors

AML

Clinical: Good prognostic factors

Bad prognostic factors

Age < 50 years > 60 years

WBC count <25,000/mm3 >25,000/mm3

FAB type M3,M4eo Mo

Auer rods present absent

Fibrosis absent absent

ALL

ALL

• Affects children more commonly• Bleeding gums, recurrent infections, tiredness• Hepato-splenomegaly• Bone marrow aspiration: lymphoblast with

PAS positive

Treatment

Chemotherapy• Remission induction : Vincristine,

prednisolone, L-asparaginase, daunorubicin• Consolidation• CNS prophylaxis: Intrathecal methotrexate+/-

CNS irradiation• MaintenanceBone marrow transplant

Prognostic factors

Clinical: Good prognostic factors Bad prognostic factors

age 4 to 10 years > 10 years, or <2 years

WBC count <50,000 mm3 >50,000 mm3

Immunophenotype B-precursor ALL Mature B or T cell

sex girls boysChromosomal number hyperdiploidy hypodiploidy

Lymphoma• Two types: Hodgkin’s and Non Hodgkins• Malignancy of the lymph nodes• Diagnosis confirmation – lymph node biopsy• Bloods plus LDH, uric acid • CXR: bihilar lymphadenopathy• Staging: CT scan chest, abdo-pelvis+BMA• B symptoms: fever, weight loss, night sweats,

lethargy

Types

• Nodular sclerosis (good prognosis)• Mixed cellularity • Lymphocyte rich• Lymphocyte depleted (poor)

Reed Sternberg Cell

• Nucleus is enclosed with an abundant amphophilic cytoplasm, and contains large, inclusion-like, owl eyed nucleoli, surrounded by a clear halo.

• Hodgkin’s Lymphoma

Ann Arbor staging

• Stage I - a single lymph node area or single extranodal site

• Stage II - 2 or more lymph node areas on the same side of the diaphragm

• Stage III - denotes lymph node areas on both sides of the diaphragm

• Stage IV - disseminated or multiple involvement of the extranodal organs

Non Hodgkin’s Treatment• Depending on subtype• Low grade if symptomless none, localized

radiotherapy, diffuse (Chlorambucil)• High grade (DLBCL) CHOP• Cychophosphamide• Hydroxydaunorubicin• Oncovin (vincristine)• Prednisolone• +/- Rituximab

Myeloma

• Malignant clonal proliferation of B-lymphocyte derived plasma cells

• Osteolytic bone lesions – unexplained backache, pathological fractures

• Anaemic, neutropenia, thrombocytopenia• Renal impairment

Investigations

• Bloods: Blood count, Hypercalcemia- increase osteoclastic activity

• Serum and urine electrophoresis• Urine sample- Bence Jones protein – K,

Lambda light chains• B2 microglobulin prognostic test• Skeletal survey- Lytic ‘punched out’ lesion

Diagnostic criteria

• Monoclonal protein band in serum or urine electrophoresis

• Increased plasma cells found on BMB• Evidence of end organ damage from myeloma - Hypercalcemia - Renal insufficiency - Anaemia - Bone lesions

Treatment

• Supportive• Chemotherapy

Complications of myeloma

• Hypercalcaemia• Spinal cord compression• Hyperviscosity• Acute renal failure

4 causes of massive splenomegaly

• CML• Malaria• Myelofibrosis• Thalassemia

EMQ questions

• A 27-year-old man presents with a two-month history of pruritis, fatigue and weight loss. On questioning he admits that whenever he drinks alcohol, he experiences bone pain. On examination he has a rubbery non-tender submandibular lymph node. He has never had infectious mononucleosis.

• A 27-year-old man presents with a two-month history of pruritis, fatigue and weight loss. On questioning he admits that whenever he drinks alcohol, he experiences bone pain. On examination he has a rubbery non-tender submandibular lymph node. He has never had infectious mononucleosis.

Hodgkin’s lymphoma

EMQ questions

EMQ questions

• A 68-year-old woman presents with a history of bruising, bone pain and lymphadenopathy. Unbeknownst to the consultant, this patient has a (t9,22) mutation known as the Philadelphia Chromosome. On examination the consultant finds a massively enlarged spleen.

• A 68-year-old woman presents with a history of bruising, bone pain and lymphadenopathy. Unbeknownst to the consultant, this patient has a (t9,22) mutation known as the Philadelphia Chromosome. On examination the consultant finds a massively enlarged spleen.

Chronic Myeloid Leukaemia

EMQ questions

EMQ questions

• A 12-year-old girl of Nigerian descent and with a known blood disorder presents to A&E with a two-day history of dyspnoea, cough and fever. You order several investigations and note that she has a Hb of 6g/dl (reference range 11.5 – 1.35 g/dl) and a chest X-ray showing pulmonary infiltrates.

• A 12-year-old girl of Nigerian descent and with a known blood disorder presents to A&E with a two-day history of dyspnoea, cough and fever. You order several investigations and note that she has a Hb of 6g/dl (reference range 11.5 – 1.35 g/dl) and a chest X-ray showing pulmonary infiltrates.

Sickle cell anaemia

EMQ questions

EMQ questions

• A 65-year-old lady presents to her GP with a 3-month history of vertigo, tinnitus and visual disturbance. She admits to feeling “a bit down” and the GP decides to carry out some routine bloods. A week later she returns and you note that her blood results show a raised high haemoglobin and a raised pack cell volume and red blood cell count.

• A 65-year-old lady presents to her GP with a 3-month history of vertigo, tinnitus and visual disturbance. She admits to feeling “a bit down” and the GP decides to carry out some routine bloods. A week later she returns and you note that her blood results show a raised high haemoglobin and a raised pack cell volume and red blood cell count.

Polycythaemia vera

EMQ questions

EMQ questions

• A 37 year old lady with known hypothyroidism presents to you with fatigue, dyspnoea and palpitations. You note that she is pale and tachycardic. Routine bloods show a macrocytic anaemia. You suspect that this is caused by her hypothyroidism. You find a positive Schilling’s test.

• A 37 year old lady with known hypothyroidism presents to you with fatigue, dyspnoea and palpitations. You note that she is pale and tachycardic. Routine bloods show a macrocytic anaemia. You suspect that this is caused by her hypothyroidism. You find a positive Schilling’s test.

Pernicious anaemia

EMQ questions

Thank you