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Hemotology
with pics
| Question | Answer |
|---|---|
| How to detect if it is an anaemia is haemolytic? | Reticulocyte (adjusted) count is up (>3%) |
| Non haemolytic normocytic anaemia? | Renal disease, Anaemias of Chronic Disease (ACD, not totally sensitive as ACD can progress to microcytic anaemia), and aplastic anaemia |
| Causes of iron deficiency anaemia and specific symptoms | Increased demand: Infants (common), Pregnancy, Decreased GI absorption, Hematuria Increased loss: Menorrhagia (common), GI bleeds VERY poor diet Glossitis, esophogeal webbing |
| Test results for iron deficiency anaemia | MCV<80, hypochromatic, Ferritin low. Ferritin VERY specific, but not sensitive (e.g. inflammation causes it to rise) |
| Causes of microcytic anaemia | Thalessaemia, Anaemias of Chronic Disease (ACD), Iron deficiancy, Lead poisoning, Sideroblastic anaemia (TAILS) |
| Pathogenesis of microcytic anaemias | Defects in heme production:Anaemias of Chronic Disease (ACD), Iron deficiancy, Sideroblastic anaemia Defects to globin production: Lead poisoning, Thalessaemia |
| Iron digestion and storage sites | Gastric acid frees Fe2+ to be absorbed. Vit C reduces Fe3+ to Fe2+ Storage from largest to smallest: Heme, Bone marrow macrophages (can be released rapidly, also present in spleen+liver), myoglobin, cofactor in enzymes |
| Intravascular Normocytic and Normochromic anaemias and general lab results | Autoimmune (cold), Mechanical (valve), GP6D deficiency, HbC, Paroxysmal nocturnal hemoglobinuria, Low haptoglobin, high Lactate dehydrogenase (LDH), damage to vasculature (valves), hemoglobinuria |
| Extravascular Normocytic and Normochromic anaemias and general lab results | Autoimmune (warm), Hereditary spherocytosis) Infection (Malaria), HbC defect, PK and GP6D deficiency, sickle cell RBC phagocytosis in the spleen. (Macrophages attack due to abnormal shape or IgG on the surface), high, UCB billirubin, high LDH |
| Intravascular Anaemia Pathology | Lysis in the blood vessel. Hb released into the blood and immediately bound by haptoglobin for clearance in the liver. LDH also released. If haptoglobin is used up the kidneys will filter. If the kidneys are maxed hb in urine. |
| Extravascular Anaemia Pathology | RBCs attacked prematurely by the liver and spleen phagocytes. Hb is converted into iron for recycling and the globine portion becomes billirubin later. LDH also, but not haptoglobin elevated. |
| Nonmegaloblastic macrocytic anaemias and pathology | DNA synthesis is unimpaired. Cause by bone marrow suppression from liver damage, alcohol abuse, metabolic disorders (purines/pyrimidines), drugs (5-FU, AZY, hydroxyurea) Reticulocytosis |
| Common pathologies in B12 and folate deficiencies | Malnutrition, malabsorbtion, Hypersegmented neutrophils, glossitis, high homocysteine. |
| Divergent pathologies in B12 and folate deficiencies | Folate: causes by impaired metabolism (MTX,Trimethoprim), higher requirements. B12: Malabsorption, tapeworm, high methylmalonic acid. Peripheral neuropathies (dorsal column first), lateral corticospinal spasticity, dementia |
| Warm autoimmune hemolytic anemia | IgG antibodies attach to a red blood cell, leaving their FC portion exposed with reactivity at 37°C. Recognized and grabbed onto by FC receptors found on monocytes and macrophages in the spleen so extravascular HA. Caused by CLL, SLE, RA, Idiopathic. |
| Cold autoimmune hemolytic anemia (Cold agglutinin disease) | At body temperatures of 28-31°C, such as those encountered during winter months in the periphery IgM bind to RBC and initiates the classical complement pathway leading to intravascular HA. Caused by Idiopathic, Lymphoma, CLL |
| Coombs direct and indirect | Direct: Anti-Human antibody to patient RBC (tests for antibodies present on RBCs) Indirect: Add potential donor RBC to patients serum. Then add Anti-Human antibody. (If there is coagulation then serum antibodies attached to donor RBC. Not suitable) |
| Plummer-Vinson Syndrome | Triad of anemia, glossitis and esophageal webs. Iron deficiency |
| Hemochromatosis | Too much Iron. Appear bronze coloured and symptom overlap with anaemia + low libido and joint pain. Primary caused my high absorption due to genetic disorders. Secondary cause by abnormal heme production from other pathologies (e.g. thalesseamia) |
| Anaemia of Chronic Disease cause + treatment | Infective/Inflammatory process -> body sequesters iron. DO NOT supplement iron. Treat the underlying problem. |
| Thalessaemia beta cause, findings, and diagnostic test | B+ allele=reduced synthesis beta globin. Bo allele=no synthesis. CoDom (Single abnormal=T minor). Microcytosis, hypochromia, target cells , poikilocytosis, EPO+ (bone scan). Minor=TC and MC sometimes Use Hb electrophoresis. Excess HbF(gamma), Hbdelta |
| Thalessaemia alpha cause, finding, and diagnostic test | 2 gene, 4 alleles. 1 delete=silent carrier. 2 deletion (cis or trans). MC and clinically similar to BT minor. 3 deletion=excess beta chains->EV hemolysis, Heinz bodies and clinically similar to BT major. Episodic pallor/anaemia. Use Hb electrophoresis. |
| B12 Metabolism | B12 is bound by R-factor which protects it from gastric acidity. In the duodenum, pancreatic enzymes hydrolyze the R-factor bond and B12 is bound by IF (which is synthesized by gastric parietal cells). The B12-IF complex is absorbed in the terminal ileum. |
| Folate Metabolism | Folate is absorbed in the jejunum. The enzyme intestinal conjugase (which is inhibited by phenytoin) is required for absorption. |
| Aplastic anaemia cause and pathology | Bone marrow failure Toxins,rarely can be due to NSAIDs, methimazole, propylthiouracil) → hypocellular bone marrow → pancytopenia Bone marrow->hypocellularity. Severe (rare): Siderosis, Macrocytic Viral: pvB19, HIV, EBV, HCV Fanconi's, radiation |
| Hereditary spherocytosis/elliptocytosis. Pathology+Findings | spherocytes, Autosomal dominant. EV HL. Normocytic with higher MCHC. Hereditary elliptocytosis: same deal but with ellipsoidal RBS. |
| Paroxysmal nocturnal hemoglobinuria | spontaneous somatic mutation of the GPI anchor necessary for CD55 and CD59 attachment. Gene on the X chromosome. GPI anchor deficiency leads to complement mediated hemolysis. IV (at night?) Sx: anemia, thrombosis, pancytopenia, hypercoagulable state |
| G6PD deficiency Pathology, Symptoms, and demographics | African+Mediterranean, X linked recessive. IV HL from increased sensitivity to oxidative stresses (no glucothione regen). Heinz bodies and Bite cells |
| Pyruvate Kinase deficiency Pathology, Symptoms, findings | autosomal recessive. Since RBCs can only produce ATP via glycolysis, this deficiency severely effects the ability of RBCs to produce energy. Decreased ATP production in the RBC → altered membrane integrity → EV HL (Splenomegaly, Jaundice) Spur cells |
| Sickle cell trait | heterozygous HbS, Protective vs Malaria. Very common in africa (1/3?) Largely asymptomatic |
| Sickle cell Anaemia Pathology | β-globin point mutation valine for glutamic acid (codon 6). Low O2 tension (higher hydrophobia interactions) → RBCs sickle and cell membranes stiffen, becoming more likely to hemolyze (EV). Treat: hydroxyurea (causes increased HbF synthesis) in severe |
| Sickle Crisis | Transportation of RBCs through inflamed tissue can also lead to occlusion of microvasculature. Then more inflammationI mediators → ↑ adhesion molecules → sickling and occlusion. and the cycle continues. Manage:NSAIDs then opiates. Spleen is special case. |
| Sickle cell secondary pathogies | AREAS Autosplenectomy, acute pain crisis Renal papillary necrosis Encapsulated organism infection (S. pneumo, Haemophilus, Salmonella etc) Aplastic anemia from Parvovirus B19 Salmonella osteomyelitis, Splenic sequestration |
| Polycythemia | Secondary: Athletes, Hypoxia. Primary:polycythemia from ectopic EPO production → normal PV, EPO up polycythemia from polycythemia vera → increased PV, EPO down |
| polycythemia vera pathology and treatment | mutation on JAK2 on chromosome 9 (myelofibrosis, myeloid metaplasia, essential thrombocythemia). Clonal expansion of myeloid stem cells (high cell count in all their lineage). hyperviscosity/phlebotomy or hydroxyurea. prognosis :6 – 18 months. ++B12 |
| Symptoms due to hyperviscosity and vessel congestion. | Hepatosplenomegaly: ++RBCs Burning pain in hands Red face Thrombosis CNS involvement Headache,Blurred vision,Retinal vein engorgement,Vertigo, ischemic attacks,Stroke ++histamine: Peptic ulcers/itching after bathing. Gout: ++cell turnover ++nucleic |
| Common Leukaemia issues | Leukopenia → infection risk Anemia Thrombocytopenia → hemorrhagic complications |
| Common symptoms/finding in AML and ALL | ACUTE onset Fever, bleeding and fatigue common Thrombocytopenia Hypercellular bone marrow, >20% blasts elevated WBC count common children and >60 years of age Without intervention, often fatal within 6 to 12 months Treat: Chemo,more rarely stem cel |
| Specific ALL pathology, finding, prognosis | Most common malignancy children. Lymphoblasts predominate B-ALL: common metastasize CNS CD10, CD19, CD20 and CD22 t(12;21) common= good T-ALL: common as ant. mediastinal mass CD1,2,3,4,7+CD10- PoorProg: Age < 2 or >12,blasts > 100K,phila chromoso |
| Specific AML pathology, finding, prognosis | 20s→early 60s as well Blasts on film APML: t(15;17) →protein w/ retinoic acid receptor and promyelocytic leuk. protein. Auer rods-> DIC t(15;17) treat w/ all-trans retinoic A, binds RARα protein, antagonist → differ. of promyelo→ neutrophils |
| Common symptoms/finding in CML and CLL | Insidious onset generalized, painless lymphadenopathy. Sometimes Hepatosplenomegaly Lab findings similar to ALs, except for blasts which are <10% in chronic leukemia. Clinical course is less devastating, but also less responsive to therapy |
| CLL demographics, pathology, findings | Most common leukemia (adults). Commonly male, > 60, Mean survival 3 to 7 years. Infiltration of marrow/hepatosplenomegaly. WBC 50-200K, warm AIHA Malignant B-cs → decreased plasma cs → hypogammaglobulinemia → ↑ infections CD5,23,19,20 translocations |
| CML demographics, pathology, findings, treatment | Peak incidence: 35-50 age Differentiated stem cs predominate Phila chrom: t(9;22) WBC 50-200K low leukocyte alkaline phosphatase activity (given WBC count) Results in blast crisis: number of blast forms ↑↑ like AML (short survival Treat: imatinib me |
| Philadelphia chromosome | ABL on c9 moves to BCR on c22 creating bcr-abl, with tyrosine kinase activity Imatinib (Gleevec) is a tyrosine kinase inhibitor specific for BCR-ABL Defines CLL, sometimes found in ALL, rarely in AML |
| Hairy cell leukaemia demographics, pathology, findings, treatment | chronic B-cell leukemia, (avg age: 55) Infiltration (marrow, etc) → Splenomegaly is more common than hepatomegaly. Pancytopenia → infections Cells stain TRAP (tartrate-resistant acid phosphatase) Responds well to therapy. Tx: 2-chlorodeoxyadenosine |
| Hodgkin's Lymphoma General Info | Young male,Reed-Sternberg (RS) cells required 4 diagnosis. Diaphoresis, pruritus, leukocytosis and localised!! nontender lymphadenopathy. ("B") signs/symptoms low-grade fever, night sweats, weight loss Disease severity = RS/Lymphocyte ratio correlated |
| Hodgkin's Lymphoma types, pathology, demographics, Prognosis | Nodular sclerosing: 65-70% male=female, fibrous bands and lacunar cps Mixed cell: 20-25% Older:Strongly associated w/ EBV LymphoC rich: 5%: 40% EBV, great pro <50males LymphoC low: 5%: HIV association, bad prog Nonclassical LymphoC rich: CD20+, CD15,3 |
| Non-Hodgkin Lymphoma Types | Periaortic nodes common Noncontiguous spread B-cells originate from germinal follicle: Burkitt's Lymphoma Diffuse large B-cell Follicular Small lymphocytic (SLL) MALToma t(1;14) T-cell lymphoma: Mycosis fungoides and Sezary syndrome |
| Non-Hodgkin Lymphoma Associated Infections | Bacterial -H. pylori: MALToma Viral Infections -HTLV-1 (Human T-cell leukemia virus type I): adult T-cell lymphoma -EBV: Burkitt's lymphoma, DLBCL, CNS lymphoma -HCV: B-cell lymphoma Hashimoto's thyroiditis predisposes to thyroid lymphoma |
| Diffuse large cell lymphoma pathology, demographics, Prognosis | most common NHL 80% B-cell in origin, 20% T-cell in origin can be present anywhere in the body. Can be found in nodal or extranodal tissue. Associated with an aggressive, widespread dissemination → rapidly fatal if untreated. CD 19,20+ |
| Small lymphocytic lymphoma | Related to CLL Older persons, indolent course B-cell origin, but most commonly express CD5 (normally a T-cell marker) and CD23. Because these are B-cells, they are also CD19 and CD20 positive Widespread nodal + liver/spleen/bone marrow also involved |
| 5yr survival rates for 4 common leukemias | ALL: 90% AML: 20% CLL: 75% CML: 90% |
| Common lymph nodes for metastatic (and primary) cancer spread. | Submental or tonsillar:SSC mouth Cervical: HeadNeck,HL L Supracla: Abdo C. R Supracla:Lung+esophageal, HL Axillary:Breast Epitrochlear:NHL, Sarcoidosis bilat) Hilar:Lung Mediastin:Lung,HL,NHL(TC) P-aortic:testicular or Burkitt Inguinal:genital |
| Splenic dysfunction and asplenia in: | - IgM, less complement activation, less C3b opsonization, more encapsulated organisms. SSHiN Salmonella, S. pnewnoniae, H. influenzae, N. meningitidis. Howell-Jolly bodies (nuclear remnants), Target cells, Thrombocytosis |
| Spleen Anatomy/Function | Long, vascular channels in red pulp with fenestrated basement membrane: filtration of RBCs. Reserve of monocytes White Pulp at centre: B cs in follicles here T cs found in tperiarterial lymphatic sheath (PALS) and white pulp Only efferent Lymphatics |
| Embryonic vs Haematological stem cells | Creates any tissue vs just blood tissue + bone? Embryo vs bone marrow origin Ethicals concern vs n.p. No phenotype vs common with CD34+ |
| GM-CSF | Granulocyte macrophage colony-stimulating factor stimulates stem cells to produce granulocytes. associated with IL4,5,13 Used to replace WBC post chemo. Also found in RA joints |
| Acute leukaemia aetiology | !Mostly unknown! Genetics(dna malfunction diseases eg Fanconi's), stem cell disorders, chemicals, radiation, cytotoxic agents (alkylating agents, topoisomerase inhibitors), viruses (HTLV->Tcell NHL) |
| EBV | Causes Infectious mononucleosis aka mono aka glandular fever. 90% of adults exposed to it Associated with HL, Burkitt's NHL, nasopharyngeal carcinoma. autoimmune diseases (dermatomyositis, SLE, RA, Sjögren's, MS |
| General side-effects of cancer therapies | Cytotoxic Radiotherapy Endocrine Immuno MonoClonal |
| Aplastic anaemia symptoms and treatment | Anaemia symptoms + purpura, mucosal bleeding, petechiae, infection Treatment: withdrawal of offending agent, immunosuppressive regimens (antithymocyte globulin, cyclosporine), bone marrow transplantation, RBC and platelet transfusion, G-CSF, or GM-CSF. |
| MGUS | Monoclonal gammopathy ofundetermined significance (MGUS) is monoclonal plasma cell expansion without the symptoms of multiple myeloma. |
| Bone cancer associated translocations | t(15;17) APML aka M3 AML. t(8;21) M2 AML inv(16) M4 AML t(4;11) ALL t(9;22) BCRABL Philadelphia chr. CLL, sometimes ALL t(11;22) Ewing's IgH Hijackers: t(8;14) Burkitts NHL c-myc t(11;14) Mantle NHL bcl-1 t(14;18) Folicular NHL bcl-2 |
| Neutropenia symptoms/signs | Tongue and GI ulcers |
| Methotrexate | |
| Chemo to test and CNS | |
| CD5 | T-cell marker. Appears on B-cells in CLL, mantle C NHL |
| Monoclonal antibodies | Highly targeting and specific antibodies. Usually end in imab |
| Rituximab | MA vs CD20. targets B-cells. Used in NHL and RA (w/ methotrexate) |
| Imatinib | aka Gleevac targets BRCABL tyrosine kinase for CML, GI tumors |
| Herceptin | aka Trastuzumab. targets Her2, a tyrosine kinase active in breast cancer. Has cardiotoxicity |
| Chimeric antigen receptor | graft IgG to TCR on T cells. Now t-cell attack self cells (according to the IgG antigen e.g. CD19) |
| Follicular Lymphoma | 25% go NHL t(14;18)→ (oncogene: inhibits apoptosis) . Overexpression of BCL2 → ↓ apoptosis. CD10,19, CD20,BCL2+,BCL6+ Lymph nodes obliterated by nodules (uniform,round,isolated) Comprised of B-cs in germinal centers of lymphoid follicles |
| Mantle Cell Lymphoma | Morphology like CLL (both CD5,19,20+) t(11;14) → bcl-1 (cyclin D1) overexertion → ↑ cell cycle → high grade; terrible prognosis avg survival 3 years |
| Ann Arbor Staging System | I: Single Lymphoid structure II: 2 or more on one side of diaphragm III: On both sides IV Extranodal involvement Also hematological involve (liver, marrow) Classically seen on PET scans |
| Langerhans cell histiocytosis | Proliferative disorder (dendritic). Dont stim T-cells with antigen presentation. S-100,CD1a,Birbeck granules 2 types: eosinophilic granuloma->pathologic fractures in adolescent HSChristianson disease->lytic lesion |
| Myelofibrosis | Fibrotic Obliteration of bone marrow. teardrop cell. in compensation Huge splenomegaly |
| Hemochromatosis | |
| Hemoglobin types | HbA: 2alpha, 2 beta HbA2: 2alpha, 2delta HbF: 2alpha2gamma Pathological Hb HbH 4 betas (severe thalassemia alpha) HbS: 2 alpha 2 betaS (SCD) HbBarts: 4 gamma (hydrops fetalis) HbC: 2 alpha 2 betaC (HbC) HbSC: HbS,SCD HBAS: 1gene SCD, 1gene SCT |
| Internation Prognostic Index | -Age > 60 -Stage 3 or 4 - 2 or more extra nodal sites - ECOG 2 or higher - LDH high 0,1 low 2 low/inter 3 inter/high 4,5 high |
| R-CHOP | Rituximab |
| Ankyrin deficiency in anaemia | Ankyrin binds CDs (55), called GPIs, -> prevent complement lysis in RBCs+platelets. ->Paroxysmal Nocturnal Hemoglobinuria. Can test CD55-. platelet lysis=thrombosis Ankyrin+spectrin, Band 3, Protein 4.2 give RBCs their shape. Def=HS 10% get AML |
Created by:
BillBillson
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