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122 Cards in this Set
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Neutrophils development |
Myeloblast Promyleocytes Myelocyte Metamyelocytes Band neutrophils Neutrophils |
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Monocytes development |
Myeloblast Promonocytes Monocytes (blood) Macrophages (tissue) |
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Opsonization |
Bacteria taken up by phagocytes |
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Function of granulocytes |
Neutrophils- bacteria Eosinophils- allergic reaction parasites Basophils- mast cells release histamine |
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Pelger huet anomaly |
Bilobe neutrophils Unsegmented neutrophils Autosomal dominant |
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May hegglin anomaly |
Neutrophils with basophilic inclusion of RNA Thrombocytopenia giant platelet Autosomal dominant |
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Chediak higashi syndrom |
Autosomal recessive Giant granules in NEML Neutropenia thrombocytopenia Heptatosplenomegaly Albinism Peripheral neuropathy |
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Leukaemoid reaction |
Excessive leukocytesosis with the present of immature cells |
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Causes of neutrophil leukocytosis |
Bacterial infection Inflammation and tissue necrosis Metabolic disease Pregnancy Neoplasm Acute hemorrhage hemolysis CML Drugs steroids lithium tetracycline |
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Cause of leucoerytgroblastic blood film |
Metastatic neoplasm in bone morrow Primary myelofibrosis Acute chronic myeloid leukemia Military TB Myeloma lymphoma Sever megaloblastic anemia Sever hemolysis Osteoporosis |
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Neutropenia |
<1.8 Blacks and Middle East <1.5 |
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Benign ethnic neutropenia |
Polymorphism of DARC gene DARC gene loss from RBC P.vivax bind to DARC gene to enter the body |
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Congenital neutropenia |
Kostmann syndrome 1st year of life Mutation of ELA2 gene |
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Drug induced neutropenia |
Anti bacterial Anti inflammatory Anti convulsants Anti thyroid carbimazol |
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Cyclic neutropenia |
Every 21 days increase monocytes decrease neutrophils |
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Clinical features of neutropenia |
Infection of Mouth and throat Painful ulcer |
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Diagnosis of neutropenia |
Bone marrow examination |
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Eosinophilia |
>0.4 >1.5 for 6 months with tissue damage hypereosinophilic syndrome Affect the heart valve CNS skin and lungs Treat steroids and cytotoxic drugs Loefflers syndrom- lungs Churhstrouss syndrom- vasculitis eosinophils granules affecting the respiratory system |
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Plasma cell |
Round Clock face Eccentric nucleus Basophilic cytoplasm Makes immunoglobulin |
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Langerhan cell histocytisis |
Bone lesion polyuria exophthalmus Eosinophilic granules 3 yes Hepatosplenomegaly lymphadenopathy eczematous skin |
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Haemophagocytic syndrome |
Autosomal recessive Associated with EBV Fever pancytopenia splenomegaly liver dysfunction Treat- corticosteroids Rituximab |
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Lysosomal storage disease |
Gaucher’s disease Tay- sach Niemann-pick |
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T cells |
T4- helper T8- cytotoxic |
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Natural killer cells |
CD 8 T cells Kill cells that have decrease HLA |
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Immunoglobulin |
2 heavy chains 2 light chains k and lambda 4IgG 2 IgA Variable V Diversity D Joining J Constant C |
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Classical pathway |
IgG IgM |
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Infectious mononucleosis |
EBV CMV Lymphocytosis due to T cells reacting with B cells B cell lymphoma CD 8 cells activated |
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Clinical features of infectious mononucleosis |
15-40 Lethargic malasis headache stiff neck dry cough Bilateral cervical lymphadenopathy Symmetrical generalized lymphadenopathy Soar throat Splenomegaly hepatomegaly 15% Follicular tonsillitis Fever |
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Diagnosis of infectious mononucleosis |
Blood film - atypical lymphocytes Increase WBC Monospot test- IgM heterophil antibody EBV antibody Cold autoimmune hemolytic anemia |
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Treatment for infectious mononucleosis |
Supportive Corticosteroids |
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Dendritic cells |
Antigen presenting cells |
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Incidence of acute myeloid leukemia |
Adults >65 |
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Acute myeloid leukemia features |
>20% blast cells CD 13 33 117 Auer rods Myeloperoxidase |
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Six groups of AML |
AML with recurrent genetic abnormalities- chromosomal translocation gene mutation Good prognosis AML with myelodisplsia related changes Therapy related myeloid neoplasia AML not otherwise specified Myeloid sarcoma Myeloid proliferation related to Down syndrome |
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Clinical features of AML |
Neutropenia thrombocytopenia anemia DIC |
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Investigation of AML |
Normocytic normochromic anemia Thrombocytopenia Increase WBC Increase blast Bone marrow hypercellular |
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Treatment of AML |
Supportive and specific Blood transfusion platelet>10 Hb>8 Induce remission <5% blast cell Cytosine arabinoside and danuorubicin Anti-33 mylotarg SCT |
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Complete remission |
< 5% blast cells Neutrophils >1 No aura rod Platelet > 100 No extramedullary disease |
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Blast cells |
1-2% normal Large punches out nucleus Abnormal accumulation of immature cells due to bone morrow failure |
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Acute promyleocytic leukemia |
T(15:17) Disruption in retinoic acid receptor Increase promyleocytes increase aura rods Aura rods cause DIC Treat- ATRA |
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Chronic myeloid leukemia |
BCL-ABL 1 Philadelphia chromosome t(9:22) ABL1 move to BCL on chromosome 22 - increase tyrosine kinase activity Chromosome 22 move to chromosome 9 Detect by FISH and reverse PCR |
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Types of polycythemia |
Absolute- red cell mass increase Relative/ pseudo polycythemia- red cell mass normal but reduce plasma volume |
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Laboratory findings in CML |
leucocytosis >200 Increase basophils and eosinophils Normocytic normochromic anemia Increase platelet Hypercellular bone morrow Increase uric acid |
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Normal hematocrit male and female |
0.52 0.48 |
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Clinical feature of polycythemia |
Old people Hyper viscosity hypervolemic hypermetbolic thrombosis Splenomegaly Plethoric appearance Conjunctival suffusion Retinal venous angorgement Gout |
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Accelerated phase of CML |
Anemia Thrombocytopenia <100 >1000 Basophils >20% Blast 10-19% Splenomegaly Several months hard to treat Transform to AML |
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Treatment for polycythemia |
HCT<4.5 Platelet <400 Venesection- decrease HCT Hydroxyuria JAK2 inhibitor- ruxoilitinib Alpha interferon Aspirin |
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Prognosis of polycythemia |
30% MF 5% AML |
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Secondary polycythemia |
Increase EPO Hypoxia- smoking COPD High altitude Venesection Aspirin |
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Essential thrombocytosis |
Increase platelet due to increase proliferation of megakayrocytes and overproduction of platelets Normal HTC |
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Diagnosis of essential thrombocytosis |
A1- platelet <450 A2- JAK2 CALR A3- no other myeloproliferative malignancy A4- no reactive thrombocytosis normal iron stores A5- bone morrow increase megakaryocytes hyperlobulted |
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Clinical features of CML |
40-60 Incidental Hyper-metabolic state Splenomegaly Anemia Thrombocytopenia Gout Visual disturbance and priapism |
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Prognosis of ET |
High risk- >60 Ply->1500 Hydroxyuria Low risk <40 Aspiring |
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Clinical feature of polycythemia |
Old people Hyper viscosity hypervolemic hypermetbolic thrombosis (bud chiari) Splenomegaly Plethoric appearance Conjunctival suffusion Retinal venous angorgement Gout Itching due to increase mast cells |
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Prognosis factor in CML |
Age Blast cell % Spleen size Platelet |
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Treatment of CML |
Tyrosine kinase inhibitor- imatinib- inhibit fusion of BCLABL1 Busutinib nilotinib Hydroxyuria Alpha interferon SCT |
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Accelerated phase of CML |
Anemia Thrombocytopenia <100 Basophils >20% Blast 10-19% Splenomegaly Several months hard to treat Transform to AML |
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Receptors of thrombocytosis |
MPL CALR |
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Myeloproliferative disease |
Clonal proliferation of one or more hemopoitic component in the bone morrow Polycythemia Vera Essential thrombotameia Primary myelobibrosis |
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JAK2 mutation |
90% polycythemia 60% ET MF |
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Polycythemia |
Increase Hb level |
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Primary myelofibrosis |
Fibrosis of the bone morrow Haemopoisis in the spleen and liver Spleenomegaly |
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Clinical features of MF |
Old people Massive spleen Hypermetabolic Bleeding problems Bone pain And gout |
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Laboratory findings in MF |
Anemia Increase WBC Leuckoerythroblastic blood film JAK2 mutation Hypercellular bone morrow Increase LDH uric acid Transform to AML |
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Treatment for MF |
Hydroxyuria JAK2 inhibitor - ruxoilitinib |
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Mastocytisis |
Increase proliferation of mast cells KIT mutation Histamine and prostaglandin Urticaria pruritis flushing abdominal pain bronchospasm |
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Myelodysplasia |
Increase bone morrow failure with dysplasia of one or more cell line Ineffective erythropoiesis Hypercellular bone morrow Pancytopenia Progress to AML |
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Cause of Myelodysplastia |
Radiation Chemotherapy |
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Incidence of myelodysplasia |
Males >70<50 |
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Clinical feature of myelodysplasia |
Cytopenia |
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Laboratory findings in myelodysplasia |
Pancytopenia Macrocytes Decrease reticulocyte Bilobe nucleus |
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Treatment of myelodysplasia |
SCT Chemotherapy |
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Hairy cell leukemia |
Proliferation of mature B cells Positive for TRAP Splenomegaly red pulp Dry tap on bone marrow aspiration Lymphadenopathy absent Males 40-60 Pancytopenia Treat- alpha interferon |
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Laboratory diagnosis in CLL |
Lymphocytosis CD 5 19 20 38 Normocytic normochromic anemia Thrombocytopenia Lymphocytes replace bone marrow Decrease immunoglobulin Autoimmune hemolytic anemia Smudge cells Increase LDH |
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Incidence of ALL |
3-7 yrs > 40 B cell 1st mutation in utero second mutation infection |
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Acute lymphoblastic leukemia |
Increase lymphoblastic in the bone morrow Children |
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Investigation of ALL |
Normocytic normochromic anemia Thrombocytopenia WBC increase/decrease/normal Increase blast cell Hypercellular bone morrow Increase uric acid LDH calcium Periodic acid schiff positive |
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Clinical features of ALL |
Neutropenia anemia thrombocytopenia Lymphadenopathy Hepatosplenomegaly Meningeal syndrome Papilloedema and hemorrhage Testicular swelling |
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Investigation of ALL |
Normocytic normochromic anemia Thrombocytopenia WBC increase/decrease/normal Increase blast cell Hypercellular bone morrow Increase uric acid LDH calcium |
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Treatment of ALL |
Supportive Blood transfusion Risk adjusted- decrease treatment in good prognosis Minimal residual disease- day 29 children month 3 adults Induce remission Consolidation Intrathecal methotrexate cytosine arabinoside steriods Maintenance- mercaptopurin and methotrexate |
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Factors for risk adjustment |
Age Gender WBC at presentation |
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Treatment for relapse ALL |
Any-CD22- epratuzimab inotuzimab Chemotherapy SCT |
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Laboratory diagnosis in CLL |
Lymphocytosis CD 5 19 20 38 ZAP70 Normocytic normochromic anemia Thrombocytopenia Lymphocytes replace bone marrow Decrease immunoglobulin Autoimmune hemolytic anemia Smudge cell Increase LDH |
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Clinical features of CLL |
Males Non tender lymph nodes cervical axilla inguinal Anemia Thrombocytopenia Splenomegaly Decrease immunoglobulin |
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Heterophile antibody |
Detectable in 2 weeks peak antibody titer- 6 weeks Paul bunnell test |
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Classification of ALL L1 |
Cell size- small homogeneous Cytoplasm- scanty Nucleus- small Nucleus size- homogeneous Basophils- variable Vacuole- variable |
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Classification of ALL L2 |
Cells size- large heterogeneous Cytoplasma- abundant Nucleus- large Nuclear size- heterogeneous Basophils- variable Vaculoe- variable |
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Classification of ALL L3 |
Cell size- large homogeneous Cytoplasm- abundant Neucleus- larger prominent Neucleus size - homogenous Basophils- present Vacuole- present |
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Classification of AML |
M0- undifferentiated M1- without maturation M2- with granulocytic maturation t(8:31) M3- acute promyleocytic t(15:17) DIC M4- granulocytic and monocytic in(16) M5 monoblastic(m5a) monocytic(m5b) M6- erythroleukaemia M7- megakaryoblastic |
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Prognosis of ALL |
Age- 2-10 vs <2>10 WBC- <10 vs >50 Gender- female vs male Type- L1/C-ALL vs L3/B-ALL Remission- early vs 4 weeks EMD- absent vs present Cytogenetic- hyperdiplody vs hypodiplody ph 11q13 CNS disease @preaentation absent vs present |
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Prognosis of CLL |
Stage - Binet A rai 0-1 vs Binet B C rai 11-IV Lymphocte doubling time - slow vs rapid Bone morrow biopsy appearance - nodular vs diffuse Chromosome - del 13q14 vs dwl 17p Genetic mutation is vs NOTcH p53 VH immunoglobulin gene hypernutated vs unmutated ZAP absent vs present CD 38 -ve vs +ve LDH normal vs raised |
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Classification of CML |
Chronic- 85% patients at diagnosis Asymptotic Accelerated Blast- >20% blast cells Choloroma 20% ALL 80% AML |
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Location of target cells |
HBC Asplenism Liver disease Thalassemia |
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Clover shape cells |
ATLL CD 3 7 25 |
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Cells in iron deficiency anemia |
Pencils cell Target cell Increase platelets |
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Calculate red cell indices |
MCV= PCV/RBC * 1000 MCH= Hb/RBC * 10 MCHC= Hb/PCV |
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Normal cell values |
Red blood cell Male- 4.5-6.5 x 10^12 Female- 3.9-5.6 Platelet- 150-400 Neutrophils 1.8-7.5 Monocytes 0.2-0.8 Eosinophils 0.04-044 Basophils 0.01-0.1 Lymphocytes 1.5-3.5 |
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Markers of AML |
CD 13 33 117 |
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Markers of ALL |
CD 10 19 20 |
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Markers of CLL |
CD 5 19 20 |
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Stages of aplastic anemia |
Sever- bone morrow cellularity <25% Neutrophils<0.5 x 10^9 Platelet <20 x10^9 Reticulocyte < 20 x 10^9 <1% Very sever- same as sever neutrophil <0.2 x10^9 Non sever - bone morrow hypocellular with peripheral blood film not meeting the sever criteria |
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Treatment of CLL |
Supportive Chemotherapy- too early shorten life expectancy CD 20- Rituximab Inbrutini- inactivate BTK cause BCell apoptosis Idealalisib- block P13k Steroids Radiation SCT |
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CLL incident |
60- 80 Europe USA B cell |
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Normal iron requirement and iron storage |
20-25 mg/kg 40-50 |
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Prognosis of Non Hodgkin’s lymphoma |
Stage Tumor size Time to complete remission Age Performance status NEMD B symptoms BM involvement |
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Laboratory prognostic factor for non Hodgkin’s lymphoma |
LDH Histology B cell vs T cell B2 microglobulin Proliferative rate |
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International prognostic index for non Hodgkin’s lymphoma |
Stage - 1/2 vs 3/4 Age <60 vs >60 Performance status 1 >1 NEMD <1 >1 LDH normal vs elevated |
1- low risk 2- low intermediate risk 3- high intermediate risk 4-high risk |
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Opportunistic infections in ATLL |
Pneumococcal jiroveci Strongyloides stercoralis Cryptococcal menigitis Crusted scabies |
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Storage pool disease |
Absence of alpha or dense granules |
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Major bleeding |
Intracranial bleeding GI bleeding Muscle bleeding- compartment syndrome Hip bleeding |
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Increase FDP |
DIC Sever liver disease Cirrhosis Thrombotic episode Surgery Trauma |
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Factor 13 deficiency |
Bleeding at birth Autosomal recessive Spontaneous bleeding Normal test Stored plasma and cryoprecipitate |
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AML unfavorably prognosis |
Age <2 >60 Cytogenetics t(8:21) t(15;17) Bad 5q7 WBC >100 M0 6 7 |
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Iron in transfused blood |
200-250 |
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Deferaxamine |
IV/SC Frequent administration Hearing loss Arthritis Growth retardation |
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Deferiprone |
Oral Agranulocytosis Nausea vomiting abdominal pain Arthritis |
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Deferasirox |
Oral Skin rash Renal failure |
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Sources of stem cell |
Bone morrow Peripheral blood stem cell Cord |
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Side effect of EPO |
CCF Depletion of iron stores Thrombosis MI stroke Headache seizures Hypertension edema |
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Paroxysmal cold hemoglobinuria |
IgG Intravascular hemolysis Red blood cell agglutinate in cold lyse in warm Viral infection syphillis predisposing cause |
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Mircoangiopathic hemolytic anemia |
DIC TTP/HUS Pre eclampsia/HELLP Mucin producing adenoma Malignant hypertension Drugs- bleomycin cisplatin mitomycin |
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Cause of death in ATLL |
Hypercalcemia Infection |
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