510 likes | 734 Vues
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.
E N D
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
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”?
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
Neutropenic Sepsis • Treat as neutropenic without waiting for FBC result • Blood cultures • Broad spectrum antibiotics within 30 minutes of presentation • IV fluid resuscitation • Get help
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?
Investigations: • Serum / urine electrophoresis • Bone marrow examination • Skeletal survey
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 • Liver Other • 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
Findings on lymph node biopsy? • Reactive • Necrotic • Granulomatous – TB, Sarcoid? • HIV? • Metastatic Carcinoma • Metastatic Melanoma • Lymphoma
Non-Hodgkin’s Lymphoma: T cell Hodgkin Lymphoma Non-Hodgkin’s Lymphoma: B cell
Case 4 • Nodular Sclerosing Hodgkin Lymphoma
Case 4 Treatment: Chemotherapy Radiotherapy • Risks of treatment: • Breast cancer • Thyroid cancer • Secondary leukaemia / myelodysplasia • Infertility • Other endocrine failure - early menopause • Bones • Cardiac damage (chemo + radiotherapy) Risks of treatment?