Presentation on theme: "Senior Academic Half Day: Malignant Haematology Beth Harrison Department of Haematology University Hospitals Coventry and Warwickshire NHS Trust."— Presentation transcript:
Senior Academic Half Day: Malignant Haematology Beth Harrison Department of Haematology University Hospitals Coventry and Warwickshire NHS Trust
Normal haematopoiesis Investigations in malignant haematology Approach to a patient with pancytopenia Diagnosis and management
Case 1 35 year old male 6 weeks recurrent throat infections 2 weeks easy bruising Hb 8.6 WCC 1.2 Platelets 12 Pancytopenia – he will need a bone marrow examination
Bone Marrow Examination
Normal Bone Marrow Aspirate
Normal bone marrow trephine
Case 1 35 year old male 6 weeks recurrent throat infections 2 weeks easy bruising Hb 8.6 WCC 1.2 Platelets 12
Hb 8.6 WCC 1.2 + Platelets 12 = Acute Leukaemia
What is acute leukaemia? What is a “blast”?
What is a stem cell?
Case 1 + Diagnosis = Acute myeloid leukaemia Bone marrow failure Blasts in bone marrow (+blood) Molecular diagnostics
Case 1 The Patient receives some chemotherapy Presents to A&E Pyrexial Shivery, vomiting, diarrhoea
Treat as neutropenic without waiting for FBC result Blood cultures Broad spectrum antibiotics within 30 minutes of presentation IV fluid resuscitation Get help
Fungal Pneumonia – Probably Aspergillus
Management of acute leukaemia Chemotherapy BUT: –Filtered air –No plants or flowers –No unnecessary visitors –Washed food – no salad or grapes or black pepper –Antifungal prophylaxis –Mouthcare
Indications for bone marrow Diagnostic –Abnormal FBC –Investigation of paraproteinaemia –Bone lesions in pelvis accessible by this route –Pyrexia of unknown origin ? TB in HIV+ ? foreign travel / splenomegaly –Isolated splenomegaly with diagnosis unclear from PB Staging –Hodgkin Lymphoma / Non Hodgkin Lymphoma Treatment response –Leukaemia, Myeloma, Lymphoma etc
Case 2 56 year old man back pain, vomiting and constipation Na 145 Calcium 3.25 K 5.7 Total protein 126 Urea 46 Albumin 34 Creat 565 Hb 8.7
Investigations: Protein electrophoresis – of what? Bone marrow examination – for what? Skeletal survey – is what?
What is the diagnosis? Multiple myeloma First management issues? Correct calcium Give fluids
Renal Failure in Myeloma Light chain deposition in kidney Hypercalcaemia Hyperuricaemia Dehydration Non-steroidal anti-inflammatories Plasma cell infiltration of kidney
Urine free light chains: An old story Previous polyclonal antisera against light chains could not distinguish light chains bound into whole immunoglobulin molecules from free light chains
Case 3 35 year old woman with 2 years of lethargy and intermittent LUQ pain now complaining of dizziness Visible white cells
Case 3 On examination: Massive splenomegaly Fundal haemorrhages Diagnosis Chronic myeloid leukaemia with hyperviscosity resulting from WCC Immediate management Get the white cell count down!!
Myeloproliferative Disorders Clonal, pre-leukaemic Uncontrolled proliferation of one or more bone marrow lineages: –Red cells – primary polycythaemia –Platelets – essential thrombocythaemia –White cells (myeloid) – chronic myeloid leukaemia –Fibroblasts - myelofibrosis
Myeloproliferative Disorders Primary Polycythaemia and Essential Thrombocythaemia: –Increased vascular events –Treatment is aimed at reducing these Hb>19? Plts>700? Ask!
Causes of hyperviscosity Paraprotein (IgM > IgA > IgG) High WCC (CML / AML > CLL) High red cell mass (polycythaemia) Raised platelet count –(>1,000, myeloproliferative rather than reactive)
Causes of splenomegaly Haematological –Chronic myeloid leukaemia, Myelofibrosis –Chronic lymphatic leukaemia –Acute lymphoblastic leukaemia –Lymphoma (various) Infective –EBV –Chronic malaria –Visceral Leishmaniasis LiverOther –HCV / HBV with portal hypertension –Any cause cirrhosis with portal hypertension
Indications for lymph node biopsy Generalised lymphadenopathy, FBC unhelpful. –(Also palpable cervical LN with mediastinal LN on CXR) Isolated lymphadenopathy – no obvious pathology in the anatomical region drained –(ENT: nasendoscopy NAD, FNA unhelpful) Regional lymphadenopathy with obvious primary pathology inaccessible to biopsy