Leukamia and Transfusion Medicine

Leukamia and transfusion
medicine
leukamia
• Blood cancer
• Acute lymphoid leukamia-T and B cell
• Acute myeloid leukamia
• Chronic lymphoid leukamia
• Chronic myeloid leukamia
Acute leukamia
• Clonal proliferation of hematopoietic stem cells
with decreased ability to differentiate into
mature elements presents with blasts in bone
marrow and periphery consequently low RBCs,
platelets, and neutrophils
• Risk factors are radiation, chemo (alkylating
agents, topo II inhib), benzene, smoking and
inherited like down syndrome,klinefelters,fanconi
• AML-peak at >65 and also 20-30
• ALL-peak at 10 years.another pick in elderly
Acute myeloid leukamia
• Peak age of 67 years
• Risk factors
– Heredity-down syndrome,hereditary
telangiectasia
– radiation
– chemical and other occupational exposures,
– and drugs e alkylating agents
• Classification is by WHO and FAB
CLINICAL PRESENTATION
• Less than 3months history due to
anemia,leucocytosis/leucopnea/leucocyte
abnormalities,thrombocytopenea
• Fatigue/weakness
• Anorexia and weight loss
• Easy bruisability/bleeding
• Fever
• Bone pain
• Lymphadenopathy
• Splenomegally
• Myeloid sarcoma-tumor mass eg on skin,lymphnode,
laboratory
• Full hemogram
– Leucocytosis,normal or leucopenea
– Normocytic-normochromic anemia commonest
– Low reticulocyte count
– Thrombocytopenea
• PBF-Auer rods(abnormal shaped rods in the cytoplasm of
the blast cells)
• Bone marrow aspirate/trephine biopsy shows a
hypercellular marrow with blasts
• Flow cytometry
• immunophenotyping
• Others-inceased uric acid,LDH,
Management principles
• Supportive management-
– optimize hemoglobin,platelets,hydration and
– manage tumor lysis syndrome- hyperuricemea/kalemea,hypoCa2+
– Proper hydration
– Treat infections –neutropenic fever
– Pneumocystitis jirovecii prophylaxis
– Psychologic support
• Definitive management
– Induction of remission-destroys bulk of the tumor through
combination chemotherapy
– Remission consolidation-after remission achieved
– Remission maintenance-after consolidation achieved
– Intrathecal chemotherapy and radiotherapy for CNS disease in ALL
• Bone marrow transplantion
Chronic myeloid leukamia
• a clonal expansion of a hematopoietic stem
cell possessing a reciprocal translocation
between chromosomes 9 and 22. the BCR-
ABL1 gene/philadelphia chromosome which
codes for cytokine kinase and oncogene
• All haematopoetic lines affected but
granulocyte affected more
• Occurs in old age peak age 55
• Has three phases
– Chronic phase-disease responsive to drugs.
– Accelerated phase-disease control more difficult
– Blast crises-disease transforms into acute
leukamia.70% AML,30% ALL
• Symptoms
– Fatigue,weight loss,lethargy,anorexia,sweating
– Abdominal pain from splenomegally
– Breathlesness,bruising
– Massive splenomegally
• Investigation
– Hemogram-leucocytosis of upto 300,000/ml with high
platelets count,normocytic anemia
– PBF-whole spectrum of neutrophils form immature to
mature.myeloblasts <10%
– Bone marrow-phase and confirm diagnosis
– Chromosome analysis
– PCR for BCR-ABL gene
• Treatment-tyrosine kinase inhibitors eg imatinib
Chronic lymphocytic leukamia
• 30% of all leukamias
• Peak age 65-70 years
• Insidious disease,diagnosis incidental
• Anemia,infections,painless
lymphadenpathy,weight loss,splenomegally
• Diagnosis-PBF showing lymphocytosis >5k
then immunophenotyping for B cell surface
antigens eg CD 19,20
lymphoma
• Maligancy of lymphoid tissue
• Hodgkins or non hodgkins
• Majority are B cell in origin
• Clinical features
– Progressive painless lymphadenopathy
– Constitutional B signs of weight loss,fever,night
sweat
– hepatosplenomegally
Investigations
• Full hemogram-may be normal or anemia

• ESR,CRP elevated
• Liver function-infiltartion of liver
• LDH-prognosis
• Chest xray-mediastinal mass
• CT chest,abdomen,pelvis-staging
• Lymph node biopsy-diagnostic
management
• Hodgkins lymphoma-reed sternberg cell
– Staging depends on number of lymph node involved
using Ann Arbor classification
– Treat using ABVD-adriamycin,,bleomycin, vinblastine
dacarbazine
• Non hodgkins-many sub type.
– Low grade- observe or chemotherapy,radiotherapy
– High grade-CHOP regimen i.e
cyclophosphamide,adriamycin,vincristone,prednisone
Multiple myeloma
• Proliferation of plasma cells which are drived
from B cells and produce immunoglobins
composed of heavy and light chains
• In myeloma immunoglobulins are monoclonal
called parapotein
• Diagnosis
– Increased serum protein on LFT suggestive
– Increased plasma cells on BMA
– Serum and urinary protein on electrophoresis
– Skeletal surve-lytic lesions on skeletal survey
– Renal dysfunction,anemia
Clinical presentation
• Bone pain from fracture,infiltration
• Normocytic anemia and very high ESR
• Hyperviscosity-cerebral ischemia,retinal
bleeds,bruising
• Renal impairment and hypercalcemea
• Spinal cord compression from vertebral
fracture or extradural mass/plasmacytoma
management
• High fluid intake to protect kidneystreat hypercalcemea
• Analgesia for bone pain
• Biphosphonates for hypercalcemea and to dela
skeletal related evenst
• Allopurinol for high uric acid
• Chemotherpay-melphalan,thalidomide and prednisone
• Radiotherapy-localised bone lesions,spinal
compression
• Bone marrow transplantation
pancytopenea
• Bone marrow failure-hypoplastic or aplastic
anemia eg inherited,drugs,viral
• Bone marrow infiltation-acute
leukamia,lymphoma,myeloma,myelodysplastic
syndrome
• Ineffective haematopoeisis-megaloblastic
anemia,HIV/AIDS
• Peripheral pooling/sequestration-hypersplenism
as in portal hypertension,felty
syndrome,myelofibrosis
• Systemic lupus erythematosus
Blood products and transfusion
medicine
• Blood is a tissue thus blood transfusion is a form
of allogenic transplant from donor to recepient
thus the risk of reactions
• Blood products
– Whole blood
– Packed cell-red blood cells
– Platelet concentrate
– Plasma-
• Coagulation factors-FFP and cryoprecipitate
• Intravenous immunoglobulins
• Human albumin
• Blood donation-low risk individuals for
HBV,HCV,HTLV,HIV
• Adverse reactions-rare but real
– Red cell incompatibility-ABO blood
groups.individuals are tolerant of their own ABO
antigens but produce antibodies against ABO
antigen they don’t have thus can mount humoral
immune response to them leading to life
threatening reaction with hypotension,shock and
renal failure.
Blood transfusion
• Blood antigens are A,B,AB and O found in plasma and
on cells
• Our bodies produce Antibodies against the antigen we
don’t have thus blood group A produces anti-B
antibody and vice-versa.blood group O produces both
anti-A/B
• Rhesus sstem is sceond mpost important.rhesus +ve is
85% of people
• 15% lack and exposure even small quantit eg
pregnancy or transfusion leads to anti-D production
• Other blood group sstems are lewis,duff,kell etc
Pre-transfusion testing
• Called grouping/screening and cross matching
to determine patients ABO and rhesus group
• Alloantibody screening identifies presence of
antibodies to major antigens in patients
• Cross matching is final step and if non
incompatibilit then that blood ok for
transfusion
Blood components
• One unit collected has about 450mls whole
blood in anticoagulants processed into
– Packed cells 180-200mls increase Hgbb 1g/dl
– Platelets 50-70mls increase b 5-10000
– Fresh frozen plasma 200-250-clotting factors
– Croprecipitate 10-15mls-factor VIII,VWF,fibrinogen
• Bed side procedure before transfusion to
ensure correctly labelled blood is given to the
correct patient
Adverse reaction to Blood transfusion
• Common but most are mild
• Vitals prior and 15minutes after transfusion
commenced to check BP,T,PR,Resp rate
• Acute hemoltic reaction=ABO incompatibility leading
to DIC,Shock,renal failure and even death is rare
• Reactions
– Febrile
– Allergic
– Acute hemolytic like ABO incompatibility
– Transfusion related acute lung injury(TRALI)
– Anaphylaxis
– Fatal hemolytic
• Infections
– HBV/HCV/HIVHTLV1/2 malaria
• Others graft versus host reaction
• Nonimmunologic reactions
– Fluid overload
– Hypothermia
– Electrolyte toxicity eg k leak from rbc causing
hyperkalemea or calcium chelated by citrate
– Bacterial contaminations-rare except in platelets
transfusion

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Leukamia and transfusion

• Full hemogram-may be normal or anemia

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