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P ointers to lymphoma and acute lymphoid leukaemia diagnosis

This article provides an overview of lymphoma and acute lymphoid leukaemia, including their presentation, diagnosis, and cardinal points in making a diagnosis. Prof. Ivy A.E. Ekem discusses the development of blood cells and the different subgroups of lymphoma and acute lymphoid leukaemia. The article also includes case studies highlighting the importance of thorough investigations in diagnosing these conditions.

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P ointers to lymphoma and acute lymphoid leukaemia diagnosis

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  1. Pointers to lymphoma and acute lymphoid leukaemia diagnosis Prof Ivy A E Ekem 16th September 2016

  2. Blood

  3. en.wikipedia.org / M. Komorniczak . Accessed 08.09.16

  4. Development of blood cells en.wikipedia.org

  5. Site of blood formation www.healthline.com>Ref Library

  6. Peripheral blood and marrow

  7. Outline • What lymphoma and acute lymphoid leukaemia are • Who they affect • How they present and why • How the diagnosis made • Cardinal points in making diagnosis ----history, examination, selected investigations and their proper interpretation

  8. What is lymphoma? What is acute lymphoid leukaemia (ALL)? • Lymphoma – cancer of a white cell in the lymph nodes. Two broad groups: • Hodgkin lymphoma • Non-Hodgkin lymphoma e.g. DLBCL, Burkitt • Acute lymphoid / lymphoblastic leukaemia - cancer of a white cell in the marrow. Three subgroups: • L1 • L2 • L3 (Burkitt type)

  9. Lymphoma • Affects lymph nodes; the spleen; thymus gland. • Lymph nodes: neck, armpits, groin, chest, abdomen and pelvis. • A lymphoma develops when an abnormal clone develops

  10. Acute lymphoid leukaemia • Affects the bone marrow • Immature lymphoid cells called lymphoblasts appear • Proliferate rapidly to populate the bone marrow and blood • Depopulate the marrow of normal cells – red cells, normal white cells, platelets • ‘Bone marrow failure’ • Lymph nodes, spleen may also be affected

  11. Same disease? • World Health Organization (WHO) classification of lymphoid tumours – listed together • Same cell, different stages of the cell at genetic change and type of change

  12. Who they affect • All ages and sexes, but in lymphoma, • Hodgkin lymphoma bimodal peak, third and eighth decades • NHL increases with age • In leukaemia • ALL is more common in childhood, especially between 2 and 5 years of age. • Risk increases again in people aged 45 and above.

  13. How they present and why • ALL • Bone marrow failure • Fever, night sweats • Enlarged lymph nodes • Lymphoma • Enlarged lymph nodes • Fever, night sweats • Unexplained weight loss

  14. Why? • Lymphoma is the most common etiology of neoplastic fever of unknown origin. • The pathophysiology: tumour necrosis factor and interleukins 1, 2, 4 • Night sweats: body temperature regulation and circadian rhythm. Periodic increases in interleukins (IL-1α, IL-2, IL-4, IL-6) and tumor necrosis factor. No research found to support this though.

  15. How the diagnosis made • History, physical examination for both • Blood film and marrow for ALL • Lymph node / tissue biopsy for lymphoma • Other tests for further classification and staging

  16. Cardinal points in making diagnosis: history, examination, selected investigations and their proper interpretation • History: Onset; suddenness in ALL. Swelling, systemic symptoms • examination: lymph node areas

  17. Lymph node areas www.healthresource4u.com

  18. Case ‘stories’ • Father insistence on marrow examination • Bone marrow trephine revealed diagnosis posthumous • Bone lesions in the young followed as for the elderly

  19. Case 1: Father insists • A 14 year old previously well BECE candidate is admitted with unexplained fever and difficulty in walking of 2 weeks duration unto the medical ward. • Examination reveals a febrile young boy, well nourished and with paraparesis

  20. Investigations among others reveal a normal blood count • Marrow is requested (by father), not obliged (re…route of request). • Father insists a friend’s son presented similarly and was found to have leukaemia • Patient seen though, but on account of normal counts……marrow not done

  21. Case 1 continued….. • Investigations continue for TB, viral illnesses etc. • LDH done • Presented at clinical meeting for leads to diagnosis • Bone marrow suggested and done on account of same history and high LDH • Diagnosis – Acute lymphoid leukaemia. Father was right.

  22. Case 2: Posthumous diagnosis • 51 year old man • Splenectomized for pancytopaenia 4 years earlier • Presented with febrile illness and severe joint pain • Managed for sepsis and rheumatoid arthritis…….improved • Pancytopaenia recurred when steroids were tapered

  23. Some FBC results • Hb – 8.1 g/dl; WBC - 3.66 x 109/l with 85%L; Platelets – 63 x 109/l • Hb - 7.9 g/dl; WBC - 5.96 x 109/l with 79.8L; Platelets – 111 x 109/l • Hb - 8.0 g/dl; WBC – 0. 88 x 109/l with 93.6%L; Platelets – 34 x 109/l

  24. Case 2 ctd…. • Hb dropping • Trephine biopsy done • Read after patients demise • Diagnosis - ALL

  25. Case 3: Bone lesions in the young • A young man, early 30s presented with features of a rapidly evolving debilitating illness • Amongst investigations, x-rays showed lytic lesions • Investigations continued as for myeloma…. • More investigations revealed an LDH of over 2000U/L • ?? Myeloma…. Patient demise • Post mortem: Lymphoma

  26. A note on Lactate dehydrogenase (LDH) • An enzyme found in nearly all living cells (animals, plants, and prokaryotes). • Catalyzes the inter-conversion of lactate to pyruvic acid and so doing converts NAD+ to NADH and back. • Raised levels in numerous medical conditions from haemolysis, through heart failure to malignancies however……

  27. LDH ctd… • Involved in tumor initiation and metabolism. • Cancer cells rely on increased glycolysis rather than aerobic respiration, thus increased lactate production • This allows tumourcells to convert the majority of their glucose stores into lactate thus shifting use of glucose metabolites from simple energy production to the promotion of accelerated cell growth and replication.

  28. The unusual presenter • Listen. Follow every lead in the history. Retake history as often as necessary • Chase every result requested for. It must have been needed for it to have been requested. Consult the laboratory physician • Interpret results with patient in mind. Remember that every patient is unique in their signs and symptoms.

  29. Take home messages for all of us • Keep learning • Don’t take patient for granted • Lymphomas and acute lymphoid leukaemias can be difficult to diagnose but • good history taking, • efficient investigations and interpretation Will give the diagnosis away in the great number of cases.

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