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2432056564630Macro-cyticMCV >10000Macro-cyticMCV >10010756909032875Folate Def-iciency00Folate Def-iciency19519909032241Aetiology: dietary (most common; especially in alcoholics); malabsorption; ? demand (pregnancy, cancer, psoriasis); dihydrofolate reductase inhibitors (eg. Methotrexate, trimethoprim); phenytoin; sodium valproateInvestigations: ?: RDW (increases early and shows there’s a variety in size of RBC’s) ?: folateManagement: 5mg folate OD; vitamin B12 if deficiency suspected00Aetiology: dietary (most common; especially in alcoholics); malabsorption; ? demand (pregnancy, cancer, psoriasis); dihydrofolate reductase inhibitors (eg. Methotrexate, trimethoprim); phenytoin; sodium valproateInvestigations: ?: RDW (increases early and shows there’s a variety in size of RBC’s) ?: folateManagement: 5mg folate OD; vitamin B12 if deficiency suspected10756906564630Vitamin B12 Def-iciency00Vitamin B12 Def-iciency19519906564631Aetiology: pernicious anaemia (autoantibodies to parietal cell and intrinsic factor ? production of intrinsic factor, prevent formation of complex; mean onset 60yrs); gastrectomy; gastric atrophy; ileal disease; pancreatic disease; malabsorption (most common cause); repeated and prolonged exposure to nitric oxide; associated with autoimmune thyroiditis, Graves, Addisons, primary hypoparathyroidism, vitiligo, myasthenia gravisSymptoms: dementia, psychosis, peripheral neuropathy, subacute degeneration of the spinal cord (occurs faster if only folate supplementation; due to demyelination; affects posterior and lateral columns); abnormal gait; gastric adenocarcinoma; carcinoid; achlorhydria; painful smooth tongueInvestigations: ?: LDH ?: B12; WBC; platelets; holotranscolabalmin Antiparietal cell and intrinsic factor antibodies; hypersegmented neutrophils; stippling of red cells; Howell Jolly bodies; Schilling test (? urinary B12 that is ? by giving intrinsic factor)Management: vitamin B12 injections; high dose PO vitamin B1200Aetiology: pernicious anaemia (autoantibodies to parietal cell and intrinsic factor ? production of intrinsic factor, prevent formation of complex; mean onset 60yrs); gastrectomy; gastric atrophy; ileal disease; pancreatic disease; malabsorption (most common cause); repeated and prolonged exposure to nitric oxide; associated with autoimmune thyroiditis, Graves, Addisons, primary hypoparathyroidism, vitiligo, myasthenia gravisSymptoms: dementia, psychosis, peripheral neuropathy, subacute degeneration of the spinal cord (occurs faster if only folate supplementation; due to demyelination; affects posterior and lateral columns); abnormal gait; gastric adenocarcinoma; carcinoid; achlorhydria; painful smooth tongueInvestigations: ?: LDH ?: B12; WBC; platelets; holotranscolabalmin Antiparietal cell and intrinsic factor antibodies; hypersegmented neutrophils; stippling of red cells; Howell Jolly bodies; Schilling test (? urinary B12 that is ? by giving intrinsic factor)Management: vitamin B12 injections; high dose PO vitamin B12246380964565Micro-cyticMCV <80? RDW00Micro-cyticMCV <80? RDW19519905760085Aetiology: hypothyroidism; vitamin C deficiencyInvestigations: normal: ferritin ?: RBC count00Aetiology: hypothyroidism; vitamin C deficiencyInvestigations: normal: ferritin ?: RBC count10763255767705Anaemia of Chronic Disease00Anaemia of Chronic Disease10756905269230MultipleMyeloma00MultipleMyeloma19519905269230Investigations: ?: globulins00Investigations: ?: globulins10756904349115Sidero-blastic00Sidero-blastic19519904348481Aetiology: hereditary ; lead poisoning; ETOH; myelodysplastic / myeloproliferative diseaseInvestigations: ?: iron; ferritin (may be normal) Normal: iron binding capacity Sideroblasts in bone marrowManagement: pyridoxine trial 2-3/1200Aetiology: hereditary ; lead poisoning; ETOH; myelodysplastic / myeloproliferative diseaseInvestigations: ?: iron; ferritin (may be normal) Normal: iron binding capacity Sideroblasts in bone marrowManagement: pyridoxine trial 2-3/1210763251948180Thal-assaemia00Thal-assaemia19519901947545Aetiology: normal globin chains produced at decreased rate haemolysisAlpha: more common in SE Asia; HbBarts (4 intrauterine death); HbH (3 moderate disease); trait (2 asymptomatic)Beta: more common in Europe; hetero minor (asymptomatic); homo major; more symptomatic than alphaSymptoms: iron overload (hepatosplenomegaly, cardiac disease); pathological #; bone deformities; paraspinal masses; endocrine failure; recurrent infectionsInvestigations: ?: reticulocytes, erythroblasts, HbF (>90%) Normal: RBC count ?: MCH; Hb (3-9) Poikilocytosis, anisocytosis, ? number of erythroid precursors on bone marrow biopsy; nucleated RBC’s; basophilic stippling; target cellsManagement: treat iron overload with desferrioxamine; stem cell transplant; often require transfusions00Aetiology: normal globin chains produced at decreased rate haemolysisAlpha: more common in SE Asia; HbBarts (4 intrauterine death); HbH (3 moderate disease); trait (2 asymptomatic)Beta: more common in Europe; hetero minor (asymptomatic); homo major; more symptomatic than alphaSymptoms: iron overload (hepatosplenomegaly, cardiac disease); pathological #; bone deformities; paraspinal masses; endocrine failure; recurrent infectionsInvestigations: ?: reticulocytes, erythroblasts, HbF (>90%) Normal: RBC count ?: MCH; Hb (3-9) Poikilocytosis, anisocytosis, ? number of erythroid precursors on bone marrow biopsy; nucleated RBC’s; basophilic stippling; target cellsManagement: treat iron overload with desferrioxamine; stem cell transplant; often require transfusions1075690964565Fe Def-iciency00Fe Def-iciency1951990964566Aetiology: GI / GU blood loss; malabsorption; pregnancy; dietaryInvestigations: ?: TIBC, transferrin ?: ferritin, iron, Hct (occurs late) MCV: Hb 10:1Management: IV iron if severe anaemia not requiring blood transfusion, or unable to tolerate PO00Aetiology: GI / GU blood loss; malabsorption; pregnancy; dietaryInvestigations: ?: TIBC, transferrin ?: ferritin, iron, Hct (occurs late) MCV: Hb 10:1Management: IV iron if severe anaemia not requiring blood transfusion, or unable to tolerate PO246380330200Anaemia00Anaemia2825751259205Normo-chromicMCV 80-10000Normo-chromicMCV 80-10011188708255000Acquired Auto-Immune Haemo-lytic00Acquired Auto-Immune Haemo-lytic20135858255000Aetiology: extravascular haemolysis due to type II hypersensitivity reaction causing RBC to be destroyed in reticuloendothelial system Warm: autoantibody vs igM on RBC in peripheries; due to lymphoproliferative infections (eg. Mycoplasma) Cold: autoantibody vs IgG / complement / both on RBC; 50% idiopathic; 50% secondary (lymphoproliferative, post-infection, autoimmune, neoplasm, connective tissue disorder, methyldopa, penicillin)Symptoms: splenomegaly; symptoms more common in warm; in cold also get Reynaud’s disease, livedo reticularis, worse in winterInvestigations: features of haemolysis; ? platelets, WBC; Coomb’s positive00Aetiology: extravascular haemolysis due to type II hypersensitivity reaction causing RBC to be destroyed in reticuloendothelial system Warm: autoantibody vs igM on RBC in peripheries; due to lymphoproliferative infections (eg. Mycoplasma) Cold: autoantibody vs IgG / complement / both on RBC; 50% idiopathic; 50% secondary (lymphoproliferative, post-infection, autoimmune, neoplasm, connective tissue disorder, methyldopa, penicillin)Symptoms: splenomegaly; symptoms more common in warm; in cold also get Reynaud’s disease, livedo reticularis, worse in winterInvestigations: features of haemolysis; ? platelets, WBC; Coomb’s positive11188707589520PK Def-iciency00PK Def-iciency20135857589520Aetiology: autosomal recessive haemolysisInvestigations: features of haemolysisManagement: partial response to splenectomy00Aetiology: autosomal recessive haemolysisInvestigations: features of haemolysisManagement: partial response to splenectomy11188706605270G6PD Def-iciency00G6PD Def-iciency20135856605270Aetiology: X-linked inherited; more common in males; black; due to oxidative stress haemolysis; triggered by infection, sulphonamides, nitrofurantoin, antimalarials, vitamin K, aspirinSymptoms: splenomegaly; neonatal jaundice; gallstonesInvestigations: features of haemolysis; G6PD assay00Aetiology: X-linked inherited; more common in males; black; due to oxidative stress haemolysis; triggered by infection, sulphonamides, nitrofurantoin, antimalarials, vitamin K, aspirinSymptoms: splenomegaly; neonatal jaundice; gallstonesInvestigations: features of haemolysis; G6PD assay20135856112510As per spherocytosis00As per spherocytosis11188706112510Ellipto-cytosis00Ellipto-cytosis11188704406265Sphero-cytosis00Sphero-cytosis20135854406900Aetiology: defect in membrane froteins (spectrin) ? deformability haemolysisSymptoms: usually asymptomatic; moderate splenomegaly (50% young children; 85% adults); jaundice; gallstones (5% children, 50% adults); haemolytic crises (usually during infections, mild); aplastic crises; leg ulcers; spinal cord lesionsInvestigations: ?: RDW????: MCHC (in 50%)Spherocytes; ? osmotic fragility; variable degree of anaemia; Coombs negative; haemolysis screen = haptoglobins, LDH, Coombs, unconjugated and conjugated bilirubin; features of haemolysis +; urinary urobilinogenManagement: splenectomy; cholecystectomy00Aetiology: defect in membrane froteins (spectrin) ? deformability haemolysisSymptoms: usually asymptomatic; moderate splenomegaly (50% young children; 85% adults); jaundice; gallstones (5% children, 50% adults); haemolytic crises (usually during infections, mild); aplastic crises; leg ulcers; spinal cord lesionsInvestigations: ?: RDW????: MCHC (in 50%)Spherocytes; ? osmotic fragility; variable degree of anaemia; Coombs negative; haemolysis screen = haptoglobins, LDH, Coombs, unconjugated and conjugated bilirubin; features of haemolysis +; urinary urobilinogenManagement: splenectomy; cholecystectomy11188704039235ARF00ARF20135854039235Investigations: Normal reticulocytes and RDW00Investigations: Normal reticulocytes and RDW11188702747010Anaemia of Chronic Disease00Anaemia of Chronic Disease20135852747010Aetiology: most common cause of normochromic; due to ? hepcidin, ? erythropoietin, inhibited marrow proliferation; altered Fe distribution; ? RBC life span; ? reticulo-endothelial activity; rheumatoid arthritis; CRF; cancer; connective tissue disordersInvestigations: ?: ferritin Normal: reticulocytes and RDW????: Fe; transferrin; TIBCManagement: blood transfusion; consider Fe; erythropoeitin00Aetiology: most common cause of normochromic; due to ? hepcidin, ? erythropoietin, inhibited marrow proliferation; altered Fe distribution; ? RBC life span; ? reticulo-endothelial activity; rheumatoid arthritis; CRF; cancer; connective tissue disordersInvestigations: ?: ferritin Normal: reticulocytes and RDW????: Fe; transferrin; TIBCManagement: blood transfusion; consider Fe; erythropoeitin11188701259205Aplastic Anaemia00Aplastic Anaemia20135851259205Aetiology: especially in SE Asia; cause by stem cell loss; idiopathic (50-65%); Fanconi’s syndrome (autosomal recessive); radiation; chloramphenicol, gold, sulphur durgs, chlorpromazine, streptomycin; viral infections (eg. Hepatitis); SLE; pregnancy; GVHDSymptoms: ? tissue oxygenation; immunocompromise; bleeding; fatigue; weakness; no splenomegalyInvestigations: ?: reticulocytes; WBC; platelets Hypocellular bone marrow; becomes macrocytic after timeManagement: supportive; antibiotics; blood products; immunosuppression; G-CSF00Aetiology: especially in SE Asia; cause by stem cell loss; idiopathic (50-65%); Fanconi’s syndrome (autosomal recessive); radiation; chloramphenicol, gold, sulphur durgs, chlorpromazine, streptomycin; viral infections (eg. Hepatitis); SLE; pregnancy; GVHDSymptoms: ? tissue oxygenation; immunocompromise; bleeding; fatigue; weakness; no splenomegalyInvestigations: ?: reticulocytes; WBC; platelets Hypocellular bone marrow; becomes macrocytic after timeManagement: supportive; antibiotics; blood products; immunosuppression; G-CSF2013585511175Alcoholism; chronic liver disease; hypothyroidism; congenital cyanotic heart disease; myelodysplastic syndromes; megaloblastic reticulocytosis00Alcoholism; chronic liver disease; hypothyroidism; congenital cyanotic heart disease; myelodysplastic syndromes; megaloblastic reticulocytosis1118870503555Other00Other282575511175Macro-cytic(cntd)00Macro-cytic(cntd)383540579120Normo-chromic(cntd)MCV 80-10000Normo-chromic(cntd)MCV 80-10012103105205095OtherHaemo-lysis00OtherHaemo-lysis20732755205095Aetiology: Infections: Clostridia, CMV, coxsackie, EBV, haemophilus, herpes simplex, HIV, malaria, measles, mycoplasma, varicella, IMN; Drugs: antimalarials, arsenic, bites, copper, lead, LA, nitrates, sulfonamides, ceftriaxone; Autoimmune: incompatible b/ transfusion, SLE, rheumatoid arthritis, ulcerative colitis, hepatitis, lymphoma, CLL; Haemolytic disease of the newborn: Rh of mother crosses placenta; need anti-DInvestigations: features of haemolysis ?: LDH, unconjugated bilirubin, reticulocytes, mild MCV, RDW, urobilinogen, faecal stercobilinogen ?: haptoglobinHeinz bodies00Aetiology: Infections: Clostridia, CMV, coxsackie, EBV, haemophilus, herpes simplex, HIV, malaria, measles, mycoplasma, varicella, IMN; Drugs: antimalarials, arsenic, bites, copper, lead, LA, nitrates, sulfonamides, ceftriaxone; Autoimmune: incompatible b/ transfusion, SLE, rheumatoid arthritis, ulcerative colitis, hepatitis, lymphoma, CLL; Haemolytic disease of the newborn: Rh of mother crosses placenta; need anti-DInvestigations: features of haemolysis ?: LDH, unconjugated bilirubin, reticulocytes, mild MCV, RDW, urobilinogen, faecal stercobilinogen ?: haptoglobinHeinz bodies12103104066540Paro-xysmal Nocturnal Hburia00Paro-xysmal Nocturnal Hburia20732754066540Aetiology: Intravascular haemolysis; Deficiency of membrane proteins injury to bone marrow stem cell pool autoimmune lysis of RBC, WBC + platelets; functional WBC defectSymptoms: Predisposition to thrombosis (venous thrombosis in 20%, often proximal); 5% become AMLInvestigations: features of haemolysis; ? WBC, platelets, iron; Hburia; urinary Fe; acidosisManagement: immunosuppression; bone marrow transplant may be curative00Aetiology: Intravascular haemolysis; Deficiency of membrane proteins injury to bone marrow stem cell pool autoimmune lysis of RBC, WBC + platelets; functional WBC defectSymptoms: Predisposition to thrombosis (venous thrombosis in 20%, often proximal); 5% become AMLInvestigations: features of haemolysis; ? WBC, platelets, iron; Hburia; urinary Fe; acidosisManagement: immunosuppression; bone marrow transplant may be curative20732753380740Aetiology: intravascular haemolysis (eg. March Hburia, cardiac valve, IABP)Investigations: features of haemolysis00Aetiology: intravascular haemolysis (eg. March Hburia, cardiac valve, IABP)Investigations: features of haemolysis12103103380739Mech-anical Trauma00Mech-anical Trauma1210310577850Sickle Cell Disease00Sickle Cell Disease2073275578486Aetiology: autosomal recessive; account for 70% congenital Hb disorders; especially in Black (occurs in 8%), Middle East, Indian; Defect in Hb production (valine/glycine) HbS polymerization when low NO concentration damage to membrane haemolysis, ? intracellular calcium, rigid RBC’s, activation of thrombosisTriggers: hypoxia, acidosis, 2,3,DPG, vascular stasis, IV volume depletion, infection, dehydration, altitude, coldSymptoms: Onset after 6 months (when HbF gone); Arthralgia, dactylitis (most common presentation <2yrs; periosteal elevation on XR, painful swelling); bone thinning; CCF; murmurs in 80%; Jaundice, gallstones, abdominal pain, Hepatosplenomegaly (spleen small in adulthood due to recurrent infarction; functional asplenia); incr bil; jaundice; gallstones; Leg ulcers; Sepsis is most common cause of death in children, especially strep pneumoniaeVasoocclusive crises (MS, GI, RS, CNS, renal), CVA, pulmonary infarction; acute chest syndromeHaematologic crises (splenic sequestration – 2nd most common cause of death, aplastic, infectious)Investigations: features of haemolysis; ? platelets, LFT’s; Hb 6-9; HbF 2-20%; RBC survival 10- 20/7 (instead of 120); reticulocytes 5-15%; Haematuria; Sickle cells; Howell Jolly bodiesManagement: see Dunn00Aetiology: autosomal recessive; account for 70% congenital Hb disorders; especially in Black (occurs in 8%), Middle East, Indian; Defect in Hb production (valine/glycine) HbS polymerization when low NO concentration damage to membrane haemolysis, ? intracellular calcium, rigid RBC’s, activation of thrombosisTriggers: hypoxia, acidosis, 2,3,DPG, vascular stasis, IV volume depletion, infection, dehydration, altitude, coldSymptoms: Onset after 6 months (when HbF gone); Arthralgia, dactylitis (most common presentation <2yrs; periosteal elevation on XR, painful swelling); bone thinning; CCF; murmurs in 80%; Jaundice, gallstones, abdominal pain, Hepatosplenomegaly (spleen small in adulthood due to recurrent infarction; functional asplenia); incr bil; jaundice; gallstones; Leg ulcers; Sepsis is most common cause of death in children, especially strep pneumoniaeVasoocclusive crises (MS, GI, RS, CNS, renal), CVA, pulmonary infarction; acute chest syndromeHaematologic crises (splenic sequestration – 2nd most common cause of death, aplastic, infectious)Investigations: features of haemolysis; ? platelets, LFT’s; Hb 6-9; HbF 2-20%; RBC survival 10- 20/7 (instead of 120); reticulocytes 5-15%; Haematuria; Sickle cells; Howell Jolly bodiesManagement: see Dunn ................
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