Haematological Disorders

Haematological disorders
• Hematopoiesis is the process by which the

formed elements of blood are produced.
• Haematopoetic stem cells are precursors of red
blood cells,white blood cells and platelets
• Growth stimulating factors affect development
and differentiation of the blood cells
– Erthropeotin-stimulate RBC production
– granulocyte –monocyte colony stimulating factors –
white blood cells
– thrombopotein -platelets
anemia
• Reduced red blood cell mass resulting in low
hemoglobin and oxygen carrying capacity
• haemoglobin
• Female more than >12-16g/dl
• Male >13.5-18g/dl
• Mean corpuscular volume-RBC size.normal is 80-
100.
• Mean corpuscular hemoglobin and mean
corpuscular hemoglobin concentration
• Reticulocyte count –Immature RBCs normal is 1-
2%.increased in blood loss or breakdown
pathogenesis
• Requirement for normal RBC formation are
– Erythropeitin-stimulate RBC production from the bone
marrow.produced by kidneys thus level drop in renal
dsease
– bone marrow –site of haematopeisis
– substrate for blood formation i.e folate,B12,iron and
other micronutrients like copper etc
• Anemia may result from abnormalities in any of
above
• Increased turnover of RBC i.e increased
breakdown causes anemia as well
Normochromic
normocytic/hypoprolferative anemia
– Anemia of chronic disease
• Infections
– Abscess
– HIV and Tuberculosis
• Connective tissue disease
– Systemic lupus erthematous and Rheumatoid arthritis
• Malignancy-bone marrow eg
Leukamia,Lymphoma,Multiple myeloma
– Anemia of chronic kidney disease
– Bone marrow infiltation-leukamia,fibrosis,other
secondary maligancy e.g prostate
Microcytic anemia
– Iron deficiency anemia

• GI bleeding
• Hematuria
• Epistaxis
• Heavy menstrual loss.pregancy
• Hookworm infestations
• Decreased absorption-sprue,crohns,increased gastric PH
– Hemolytic anemia
• Sickle cell disease
– Sideroblastic anemia
Macrocytic anemia
• Macrocytosis but no megaloblastosis
– Hypothyroidism
– Liver disease
– Alcoholism
• Megaloblastic anemia
– Folate deficiency
– Vitamin B 12 deficiency
Clinical manifestation
• Due to reduced oxygen delivery to tissue
– Fatigue
– Exertional dyspnea
– Angina
– Pedal edema
• Signs
– Palor
– Tachcardia and Hypotension in acute blood loss
– Jaundice-hemolysis,
– Splenomegally-leukamia,chronic hemolysis,hemolysis
• Petechiae/purpura-bleeding disorders
• Glossitis-iron,folate,B12 deficiency
• Lymhadenopathy in lymphoproliefeative
disorders
• Koilonychia-iron deficiency
• Neurologic deficiency-folate/b12 deficiency
Approach to patient with anemia
• History
– Signs and symptoms
– Melena stool or frank GI blood loss
– Menstrual history
– Alcohol history
– Family history/ethnicity.geographical history-sickle
cell in western,nyanza and coast
– Drugs and medication history
– Associated symptoms of fever,nightsweats
laboratory
• Full blood count
– Hemoglobin,haematocrit,MCV/MCH,MCHC
– Other cells count-platelets,WBC
– Reticulocyte count-high in blood loss
• Peripheral blood film-size,shape of RBC
• Renal function test-low erthropoetin in CKD
• Iron studies-serum iron,total iron binding
capacity,transferrin and ferritin levels
• Serum folate,B12 levels
• Bone marrow aspirate and biopsy in leukamia
treatment
• Depends on cause
• Replacement of iron,folate,B12
• Erythropoetin in chronic kidney disease
• Management of hemolysis e.g hydroxyurea in
sickle cell,prednisone in autoimmune
hemolysis
polycaethemia
• increase in the hemoglobin above normal
• Relative if cause is decreased plasma volume
e.g dehydration
• Absolute is increase in RBC mass
– Male HGB >18g/dl,haematocrit >50%
– Female >16g/dl,haematocrit >45%
• History of smoking,high altitude
residence,athletes,chronic lung
disease,congenital heart diseases
• Presentation depends on underlying cause if
secondary or hyperviscosity and thrombosis
• Thrombosis-digits,budd-chiari,abdominal vessels
thrombosis is common
• Hyperviscosity-vertigo,tinnitus,headache,visual
disturbance,hypertension
• Polycythaemia-aquagenic pruritus and symptoms
related to hepatosplenomegaly
• Easy bruising,bleeding from GI,peptic ulcer
disease
Laboratory
• Full blood count-hemoglobin and haematocrit
levels
• Erythropoetin-
– if low it suggest polycythemea vera
– If high it could be physiologic response to
hypoxemic disorders like chronic lung disease or
cyanotic heart disease.
– autonomous production of EPO e.g
hepatoma,fibroids,renal cell carcinoma
Haemolytic anemia
• Intrinsic mechanisms
– Enzyme deficiency e.g Glucose 6 phosphatase
deficiency,pyruvate kinase
– Membrane abnormalities-hereditary
spherocytosis,elliptocytosis
– Hemoglobinopathies-sickle cell disease,thalasemia
• Extrinsic
– Traumatic-prothetic valves,microangiopathic hemolytic
anemia
– Immune mediated-autoimmune,drugs,
– Direct infections e.g malaria
– Entrapment -hypersplenism
diagnosis
• Low hemoglobin
• Increased reticulocyte count and LDH
• Decreased haptoglobin as it binds bilirubin
• Increased indirect bilirubin
• Autoimmune-positive coombs test
• Intravascular hemolysis-
– increased reticulocyte count,
– low haptoglobin,hemoglobinuria
• Extravascualr-
– splenomegally
• Family or self history of cholelithiasis
Sickle cell disease
• Autosomal recessive disorder with glutamate
substitution for valine at 6 amino acid on beta globin
chain
• Structurally abnormal hemoglobin that polymerises
when deoxygenated forming sickle with poor deformity
and thus hemolysis and occlusion of microvasculature
• Vaso-occlusion and infarction
• Painful crises-hands and feet syndrome
• Acute chest syndrome
• Cerebrovascualr accidents
• Renal papillary necrosis
• priapism
– Infections
• Splenic infarction leading to infection with capsulated
organisms eg streptoccus,haemophylis
• Osteomyelitis-infarcted bone salmonella
• Diagnosis
– Sickle shaped RBC and howel jolly bodies
– Low hemoglobin and MCV
– HB electrophoresis
– Sickling test-historical
management
• Hydroxyurea
– Increase HBF(fetal hemoglobin) that doesn’t sickle
and releases oxygen easily
– Reduce painful crises,acute chest syndrome and
mortality
• Supportive
– Folate to prevent deficiency from high cell
turnover
– Vaccines-
pneumococcal,meningococcal,H,influenzae,HBV
management
• Acute painful crises
– -hydration at 1.5-2 normal fluid requirements
– Analgesic with opiods,NSAIDS,paracetamol
– Oxygen
• Blood transfusion,simple transfusion or
exchange transfusion in stroke/TIA or acute
chest syndrome,
• pre-op target HGB 10
• Stem cell transplant is curative
Other hemolytic disorders
• Hereditary spheroctosis and elliptoctosis-
defective cytoskeleton
• Glucose 6 phosphatase dehdrogenase deficency
-increased susceptibility to oxidative stress e.g on
exposure to drugs e.g quinine,DKA,fava beans
• Microangiopathic hemolytic anemia-
HUS,TTP,eclampsia
• Autoimmune hemolytic anemia
• Drug induced-antibody mediated eg
cephalosporin,sulfur
drugs,methyldopa,NSAIDS,sulfonylurea etc
splenomegally
• mechanism and underlying etiology are
– Reticuloendothelial hyperplasia
• Hemolytic anemia
• Thalasemea
– Immune hyperplasia
• Infection e.g EBV,CMV,TB,kalazaar,typhoid,MAC
• Autoimmune disease-SLE,Rheumatoid arthritis(felty
syndrome)
– Congestion
• Portal hypertension,congestive cardiac failure
• Cirrhosis/shistosomiasis
• Portal vein thrombosis
splenomegally
• Infiltration/non malignant
– Lysosomal storage disorders-gauchers
– Splenic cysts
• Neoplasm
– Myeloproliferative disorders e.g chronic myeloid
leukamia,primary myelofibrosis,polythemia vera,
– Acute leukamia-ALL,AML
– Lymphoma-non hodgkins,hodgins,chronic
lymphocytic leukamia
lymphadenopathy
• Size-if less than 1cm x 1cm likely benign
• Description-soft, firm, rubbery, hard, discrete,
matted, tender, movable, or fixed
• Leukamia/lymphoma-large, discrete, symmetric,
rubbery, firm, mobile, and non tender
• Metastatic tumor-hard, nontender, and
nonmovable because of fixation to surrounding
tissues
• Virchow's node is an enlarged left supraclavicular
node infiltrated with metastatic cancer from a
gastrointestinal primary
Localised lymphadenopathy
• Location nodes may provide clues
– Supraclavicular and scalene nodes almost always
pathologic e.g Virchows in GIT
malignancy,TB,lymphoma
– Neck nodes-URTI,head and neck tumors
– Axillary-upper arm pathologies or breast
– Inguinal-infection of lower limbs,melanoma,STDS
eg chancroid,syphillis,LGV,metastatis from
pelvis,rectum
Generalised lymphadenopathy
• Infectious diseases
– Viral-EBV,CMV,hiv
– Bacteria-TB,salmonella,syphillis
– Parasite-leishmaniasis,filariasis,trypanosomiasis
– fungal,-rare
• Connective tissue disease-R.arthritis,SLE,
• Drugs sensitivity-phenytoin,hydrallazine
• Malignancy-
leukamia,lymphoma,breast,intabdominal,lung tumors
• Lipid storage disorders

Haematological Disorders

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Haematological disorders

• Hematopoiesis is the process by which the

– Iron deficiency anemia

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