Introduction to Haematological Diseases

• Redness in angles of lips - sore & uncomfortable. Seen in pts with severe iron deficiency.

  
• Iron deficiency anaemia is indicated by red blood cells that are paler and of a smaller size than normal.

  
• Causes of iron deficiency:

  • Dietary deficiency
  • Malabsorption
  • Blood loss
  • GI tract
  • Menorrhagia (heavy menstrual bleeding)
• B12 and folate deficiency leads to macrocytic anaemia:

  • Only 4 months supply of folate in the body, found in leafy vegetables, fruit and liver. Absorbed in small bowel.
  • 2-6 years supply of B12 in body, found in animal protein. Absorption complex, needs intrinsic factor. Absorbed in terminal ileum.

  
• Macrocytic anaemia (caused by B12 and folate deficiency) is identified by hypersegmented neutrophils with large red cells.

  
• Causes of folate deficiency:

  • Intake - eg elderly pts
  • Requirements - eg pregnancy, haemolysis
  • Malabsorption - eg coeliac disease
  • Alcoholism
  • Drugs - eg Septrin, Methotrexate
• Causes of B12 deficiency:

  • Inadequate intake...rare in very strict vegetarians...no milk or eggs
  • Low gastric acid (10-30% patients with partial gastrectomy)
  • Intrinsic factor/antibodies against parietal cells (pernicious anaemia)
  • Abnormal terminal ileum
• Diagnosis of B12 and folate deficiency:

  • Red cell folate levels
  • Serum vitamin B12 levels, intrinsic factor antibodies
  • Treatment - identify underlying cause, oral folate replacement or lifelong injections of Vitamin B12 (3 monthly)
• Other causes of macrocytic anaemia:

  • Drug-related - methotrexate
  • Alcohol
  • Myelodysplasia
• Causes of petechiae:

  • Due to reduced number of platelets
  • Most common cause: immune thrombocytopenia (ITP) - patients develop antibodies against platelets
  • Causes of disseminated intravascular coagulation (DIC)
  • Recent infection - mononucleosis etc
  • Bone marrow infiltration - acute leukaemia
  • Bone marrow failure syndromes - aplastic anaemia

  
• In aplastic anaemia most of the bone marrow space has been replaced with fat. No normal haematopoiesis taking place.

  
• Acute leukaemia:

  • Present with short history bruising bleeding/infection, sometimes skin rash
  • Lymphadenopathy/gum hypertrophy
  • Can be life threatening
  • Treatment:
  • Depends on subtype and cytogenetics
  • Most patients treated in clinical trials
  • May include bone marrow transplantation or chemotherapy
  • Prognosis 30-70% at five years depending on leukaemia type and treatment option
  • Prognosis much poorer in the elderly

  
• Osteonecrosis of the jaw:

  • Associated with bisphosphonates
  • Difficult to treat
  • Severe bone disease affecting the jaws leading to necrosis probably ischaemia related
  • Nitrogen-containing bisphosphonates worse specially pamidronate and zoledronate
  • Important: if dental extraction or root canal treatment required stop bisphosphonates; give antibiotics - augmentin for example
• Myeloma:

  • Nearly all myeloma pts are on bisphosphonates
  • Median age of presentation = 67
  • Presents with symptoms of anaemia, bone pain, infection or constipation, stomach upsets secondary to hypercalcaemia
  • Lab findings: normochromic normocytic anaemia, paraprotein, Bence Jones proteinuria, high viscosity or ESR
• Non-Hodgkin's lymphoma:

  • More common than Hodgkin's lymphoma
  • More than 100 different subtypes
  • Classification system has changed several times
  • Treatment
  • Depends on age and subtype
  • May be anything from 'careful observation' to innocuous oral chemo to intensive chemotherapy and transplant
  • Outcome:
  • High grade lymphomas are curable, but lethal if not cured
  • Low grade lymphomas are typically not curable but controllable for long periods (several years)
• Mucositis is inflammation of the mucosal surfaces throughout the body. It typically involves redness and ulcerative sores in the soft tissues of the mucosa. Oral mucositis manifests as erythema, inflammation, ulceration, and haemorrhage in the mouth and throat. Frequently seen post chemotherapy and radiotherapy.
• Oral candidiasis: treat with nystatin and in severe cases with oral fluconazole.

  
• For herpes simplex cold sores, consider to treat with topical acyclovir - reactivations common.

  
• Herpes zoster - shingles:

  • Reactivation of chicken pox
  • Dermatomal distribution
  • Can be very painful
  • Treat with oral acyclovir