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81 Cards in this Set
- Front
- Back
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DDX Neutropenia
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Myelophthisic Anemia
Aplastic Anemia Large Granular Lymphocytic Leukemia Defective DNA Hypercellular Bonemarrow Pancytopenia with abnormal WBC/RBC morphology B12 or Folate deficiency myelodysplastic Syndromes 2ndry to SLE/Antibodies Rx: Dose related (CA Rx), Ideosycratic, Immunologic, ETOH Overwhelming Infections Splenic Sequestration 2ndry to enlarged spleen Felty Syndroem: RA, Splenomegaly Neutorpenia |
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Myelocyte
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Granulocyte
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IL-5
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Promotes Eosinophil Lineage
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Myeloblast
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Earliest Recognizable Cell Differentiated along Myeloid lineage (Granulocytes, platelets, erythrocytes)
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Maturation progression of granulocytes
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Blasts
Promyelocyte Myelocyte Metamyelocyte Band Neurtophil Segmented Neutrophil |
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Granulocytopenia
Clinical Manifestations |
<1500 granulocytes
a reduction in all lines of granulocytes, but really neutrophils Lots of causes but that's not an objective Clinical Manifestations --Agranulocytic Angina: Ulceration of mucosa <1000 = increased risk for infection --Risk for invasive infxn, esp Aspergillus & Candida |
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Ulceration of lateral tongue
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Agranulocytic Angina,
Ulceration of all UG/GI Mucosa possible when Neutrophils <1500 |
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Agranulocytic Angina
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Ulceration of UG/GI Mucsoa probable when neutrophils <1500
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Invasive Aspergillus
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Common Infection with Neturopenia
Neutropenia defined as <1500 Infections become invasive <1000 |
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DDX Pancytopenia
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Reduced RBC's, WBC's and Platelets
>>Acute Leukemia<< Call the Hematologist, Do a Bone Marrow Today Other Myelophthisic Anemias Megaloblastic Anemia Aplastic Anemia Autoimmune Disease eg SLE Myelodysplastic Syndromes Splenic Sequestration Drugs, eg CA Chemotherapy High Dose Total Body Radiation |
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Lymphocytopenia
Definition, Causes |
Absolute Lymphs <1200 in adults, <3000 in children
Causes: 1. Acquired immune deficiences HIV 2. congenital T cell immune deficiencies (DiGeorge) malnutrition Corticosteroids: lysis TB/Disseminated Granulomas Viral Infections (exiting blood) Radiation SLE |
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Causes of Neutrophilia
"Memorize this List" |
Bacterial Infections, Mildly & Transiently with Viral Infections
Inflammatory: Rheumatic Fever, Vasculitis Neoplasia, Hemorrhage Drugs: glucocorticoids, lithium, Colony Stimulating Factors Metabolic: acidosis, uremia, gout, thyroid storm Tissue Necrosis: infarction, trauma, burns, surgery Misc: excercise, giving birth Decreased Mragination: exercise, catecholamines-->rapid increase in mature (segmented) neutrophils Increased Release from Bone Marrow: Acute Infxn, Sterile Inflam +Hypoxia (MI), endotoxin;-->Inflam Cytokines TNF, IL-1--> increased numbers of bands Growth Factor mediated Increased Marrow Prodxn- Req's Days of chronic inflam or paraneoplastic growth factor Growth Factor Independent Marrro Prodxn- Neoplasms= blast cells Decreased extravasation into ts (corticosteroids) |
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What morphologic changes are seen in neutrophils during severe inflmmation?
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Toxic Granulation
Coarse darker than normal cytoplasmic granules, persistence of primary granules Dohle Bodies: blue staining dilated endoplasmic reticulum Cytoplasmic Vaculoles |
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Toxic Granulation
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Coarse darker than normal cytoplasmic granules, persistence of primary granules in Neutrophils
Means they were rushed out of the factory = severe inflam, probably bacterial infxn |
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Dohle Bodies
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blue staining dilated ER in Neutrophils
Means they were rushed out of the factory = severe inflam, probably bacterial infxn |
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Cytoplasmic Vacuoles in Neutrophils
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means they were rushed out of the factory = severe inflam, probably
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Leukemoid Reaction
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High granulocyte counts
Typically ~50k From inflam, infection, tumors, etc |
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Left Shift
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increased immature granulocytes= bands, metamyelocytes
From inflam, infection, tumors, etc |
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Leukoerythroblastic Reaction
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presence of nucleated RBC's and immature neutrophils in peripheral blood smear
usually a space occupying lesion: malignancy myeloproliferative disorder granulomas gaucher disease |
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What causes Eosinophilia?
What do you see on biopsy? What is the Tx, what are the side effects? |
IL5 stimulation driving a Type 1 hypersensitivty reaction:
most likely in US allergies Rest of the DDx: Rx reactions parasitic disease skin disease some malignancies eg Hodgkin collagen vascular disorders vasculitides (Churg-Strauss) Eosinophlic Pneumonias: Cocy, Loeffler's, Chronic Chacot-Leyden Christals May be reduced with corticosteroids, will also lyse lymphocytes, will increase neutrophils (cannot diapedese) |
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Charcot Leyden Crystals
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Precipitated Byproduct of Eosinophilic Granules
indicative of asthma |
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What are the causes of monocytosis
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Recovery from Acute Infxn
Anything granulomatous Chronic Infxns: Subacute Bacterial Endocarditis TB Malaria, Rickettsia, Brucelosis Collagen Vascular Disease: SLE, RA Inflammatory Bowel Disease Recovery from Acute Infxn Hodkin & Some T cell Lyphomas |
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What are the causes of lymphocytosis
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Pertussis (Whooping cough)
Chronic bacterial infxn (TB) Viral infxn With Reactive/Atypical Lymphs (CD8+) Viral infection: infectious mononucleosis most likely Dx if reactive lymphos >20% CMV IM like w/o cervical nodes Toxoplasmosis Viral Hepatitis >4k in adults lymphoproliferative neoplasla most commonly CLL |
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What is the significance of atypical lymphs
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Activated CD8's, Indicative of viral infection
infectious mononucleosis most likely Dx if reactive lymphos >20% Confirm mononucleosis with monospot test for heterophile antibodies CMV IM like w/o cervical nodes Toxoplasmosis Viral Hepatitis |
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What hematologic conditions other than lymphocytosis may be associated with infectious mononucleosis?
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10% will have autoimmune phenomenon
cold acting antibodies thrombocytopenia |
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Nucleated RBC's DDX
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Do not appear in normal peripheral blood
Rapidly evolving anemias when Hb< 5g Post Splenectomy Myelophthisic bone marrow replacement |
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Hairy Cell Leukemia
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CD19, CD20, + Monocyte associated antigens CD11c & CD22
Post-germinal center B memory cell Formerly Dx'd by TRAP stain marrow replacement, marrow: dry tap-->pancytopenia; monocytopenia-->mycobacteiral infxns, fevers LN's normal, splenomegaly from hematopoiesis prognosis for survival excellent |
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CD19, CD20, CD11c & CD22
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Hairy Cell Leukemia
Post-germinal center B memory cell Formerly Dx'd by TRAP stain marrow replacement, marrow: dry tap-->pancytopenia; monocytopenia-->mycobacteiral infxns, fevers LN's normal, splenomegaly from hematopoiesis prognosis for survival excellent |
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Post-germinal center B memory Cell leukemia
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Hairy Cell Leukemia
CD19, CD20, + Monocyte associated antigens CD11c & CD22 Formerly Dx'd by TRAP stain marrow replacement, marrow: dry tap-->pancytopenia; monocytopenia-->mycobacteiral infxns, fevers LN's normal, splenomegaly from hematopoiesis prognosis for survival excellent |
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Myelodysplastic Syndromes vs Myeloproliferative Disoders
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Myelodysplastic Syndromes- precancer of the myeloid lineage, not enough cells in the peripheral blood
myeloproliferative- precancer of the myeloid lineage with too many cells in the peripheral blood |
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Chronic Myeloproliferative Disorders
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Clonal transofrmation of a multipotent myeloid progenitor cell
(except CML wherein it is a pluripotent stem cell features: increased marrow cellularity due to increased hematopoiesis Splenomegally; extramedullary hematopoeisis may terminate in marrow fibrosis may transofrm into AML |
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Polycythemia Vera
Pathogenesis, Lab, Clinical Features, Complications Cause of Death |
Mutation of JAK2: Neoplasm of multipotent myeloid stem cell leading predominantly to erythrocytosis, but also with granulocytosis and thrombocytosis
Splenomegaly also present initally due to congestion but ultimately a byproduct of extramedullary hematopoiesis Hypercellular bone marrow with normal M:E ratio Plethoric (excess fluids), cyanotic complexion (stagnation/doxygenation) Splenomegaly, HTN, HA, hypermetabolic-->gout Peptic ulceration and intense pruritis from basophils Stagnant flow--> platelet dysfnx-->risk of bleeding Most common cause of death is thrombosis |
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Plethoric & cyanotic complexiion
Splenomegaly, Htn, HA Peptic Ulcers, Intense Pruritis |
Polycythemia Vera
Mutation of JAK2: Neoplasm of multipotent myeloid stem cell leading predominantly to erythrocytosis, but also with granulocytosis and thrombocytosis Splenomegaly also present initally due to congestion but ultimately a byproduct of extramedullary hematopoiesis Hypercellular bone marrow with normal M:E ratio Stagnant flow--> platelet dysfnx-->risk of bleeding Most common cause of death is thrombosis |
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Myelodysplastic Syndromes
Define, Natural Hx, Morphology in Bone & Blood |
Group of clonal stem cell disorders charactrized by ineffective hematopoiesis resultin in cytopenias, progeny are dysfnx
"Pre-leukemia" Risk for AML Males over 50 with insidious onset Karyotype correlates with behavior 5q- is the best prognosis Pancytopenia, Poikilocytosis, Gypocellular granulocytes or toxic granulation, pseudo pelger huet cells (binucleated granulocytes), giant platelets Bone usually hyperceullar; megaloblastoid maturation, ringed sideroblastgs [shit this is huge] |
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Pancytopenia, Poikilocytosis, Hypogranular granulocytes or toxic granulation, pseudo pelger huet cells, giant platelets
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Myelodysplastic syndrome: "Pre-leukemia" Risk for AML.
Group of clonal stem cell disorders charactrized by ineffective hematopoiesis resultin in cytopenias, progeny are dysfnx Males over 50 with insidious onset Karyotype correlates with behavior 5q- is the best prognosis Pancytopenia, Poikilocytosis, Gypocellular granulocytes or toxic granulation, pseudo pelger huet cells (binucleated granulocytes), giant platelets Bone usually hyperceullar; megaloblastoid maturation, ringed sideroblasts |
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;pseudo-Pelger-Huet Cells
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binuclated granulocytes, seen in Myelodysplastic syndrome: "Pre-leukemia" Risk for AML.
Group of clonal stem cell disorders charactrized by ineffective hematopoiesis resultin in cytopenias, progeny are dysfnx Males over 50 with insidious onset Karyotype correlates with behavior 5q- is the best prognosis Pancytopenia, Poikilocytosis, Gypocellular granulocytes or toxic granulation, pseudo pelger huet cells (binucleated granulocytes), giant platelets Bone usually hyperceullar; megaloblastoid maturation, ringed sideroblasts |
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Essential Thromobocytosis
Define most common genetic findign frequency clinical course |
Uncommon Myeloproliferative Disorder
>600k w/o features of other MPD's JAK2 mutation Dx of exlusion with reactive thrombocytosis large dysfnx platelets increased megakaryocytes in bone marrow thrombosis & hemorrhage w/ indolent course, survival 12+ years |
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too many platelets, too large on smear
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Essential Thromobocytosis
Uncommon Myeloproliferative Disorder >600k w/o features of other MPD's JAK2 mutation Dx of exlusion with reactive thrombocytosis large dysfnx platelets increased megakaryocytes in bone marrow thrombosis & hemorrhage w/ indolent course, survival 12+ years |
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Reactive Thrombosis DDx
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Acute Infxn, Inflam
Chronic Inflam Asplenism Acute Blood Loss/Hemolysis Iron Deficiency Anemia Solid malignancies initial response to myelophthisic process rebound after myelosuppression |
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Primary myelofibrosis
morphology, lab, clinical |
Clonal neoplastic transformation of multipotent myeloid stem cell causing fibrosis in bone marrow and myeloid metaplaisa: hematopeisis in other organs.
60+ yo w/ JAK2 mutation, DDx: myelophthisic anemias Neoplastic megakaryocytes release PDGF, TGF-B ressuling in fibrosis of bone marrow and suppression of normal hematopoeisis Dysfnx RBC's, WBC's, platelets massive splenomegaly, hyperuricemia, fatigue, weight loss, night sweats Death w/in 5 years by infxn, bleeding/thrombosis, transformation to AML in LN or Spleen Normocytic, Normochromatic Anemia, Leukoerythroblastosis with Left Shift an NRBC's. tear drop RBCs w/ basophilic stippling (iron poisoned ER) "Howel Jolly Bodies" |
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Tear drop erythrocytes
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Primary myelofibrosis: Clonal neoplastic transformation of multipotent myeloid stem cell causing fibrosis in bone marrow and myeloid metaplaisa: hematopeisis in other organs.
60+ yo w/ JAK2 mutation, DDx: myelophthisic anemias Neoplastic megakaryocytes release PDGF, TGF-B ressuling in fibrosis of bone marrow and suppression of normal hematopoeisis Dysfnx RBC's, WBC's, platelets massive splenomegaly, hyperuricemia, fatigue, weight loss, night sweats Death w/in 5 years by infxn, bleeding/thrombosis, transformation to AML in LN or Spleen Normocytic, Normochromatic Anemia, Leukoerythroblastosis with Left Shift an NRBC's. tear drop RBCs w/ basophilic stippling (iron poisoned ER) "Howel Jolly Bodies" |
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Massive Splenomegaly, Hyperuricemia, Fatigue, Weight Loss, Night Sweats
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Primary myelofibrosis: Clonal neoplastic transformation of multipotent myeloid stem cell causing fibrosis in bone marrow and myeloid metaplaisa: hematopeisis in other organs.
60+ yo w/ JAK2 mutation, Dysfnx RBC's, WBC's, platelets Neoplastic megakaryocytes release PDGF, TGF-B ressuling in fibrosis of bone marrow and suppression of normal hematopoeisis massive splenomegaly, hyperuricemia, fatigue, weight loss, night sweats Death w/in 5 years by infxn, bleeding/thrombosis, transformation to AML in LN or Spleen Normocytic, Normochromatic Anemia, Leukoerythroblastosis with Left Shift an NRBC's. tear drop RBCs w/ basophilic stippling (iron poisoned ER), "Howel Jolly Bodies" DDx: myelophthisic anemias |
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Review and recognize sources of occupational and environmental lead exposure.
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ha!
paint, go with paint |
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Howell Jolly Bodies
This card is not correct |
Basophilic Stippling in RBC's remnant of iron poisoned ER
Seen in Primary myelofibrosis: Clonal neoplastic transformation of multipotent myeloid stem cell causing fibrosis in bone marrow and myeloid metaplaisa: hematopeisis in other organs. 60+ yo w/ JAK2 mutation, Dysfnx RBC's, WBC's, platelets Neoplastic megakaryocytes release PDGF, TGF-B ressuling in fibrosis of bone marrow and suppression of normal hematopoeisis massive splenomegaly, hyperuricemia, fatigue, weight loss, night sweats Death w/in 5 years by infxn, bleeding/thrombosis, transformation to AML in LN or Spleen Normocytic, Normochromatic Anemia, Leukoerythroblastosis with Left Shift an NRBC's. tear drop RBCs w/ basophilic stippling (iron poisoned ER--"Howel Jolly Bodies") DDx: myelophthisic anemias |
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Consequences of Lead Exposure
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30 year halflife, radiodense desposits in bones and teeth
blocks iron into heme: ferrochelatase & delta aminolevulinic acid dehydratase hypochromic microcytic Anemia w/ basophilic stippling Competes with Ca2+, Impaired 1, 25 DH vitamin D metabolism interferes with normal remodelling of epiphysial cartilage in children inhibits healing of fractures Inhibits membrane-associated enzymes hemolysisis renal damage-->htn, gout demyelination:-->wrist drop, ankle drop,--> Low IQ in children GI Sx, Oral Lead Line, Interfility, Delayed Puberity in Girls |
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Good Chart on page 276 of WBC intro
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DDx of anemia
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HA, Memeory Loss
Wrist Drop, Foot Drop |
Consequences of Lead Exposure
30 year halflife, radiodense desposits in bones and teeth blocks iron into heme: ferrochelatase & delta aminolevulinic acid dehydratase hypochromic microcytic Anemia w/ basophilic stippling Competes with Ca2+, Impaired 1, 25 DH vitamin D metabolism interferes with normal remodelling of epiphysial cartilage in children inhibits healing of fractures Inhibits membrane-associated enzymes hemolysisis renal damage-->htn, gout demyelination:-->wrist drop, ankle drop,--> Low IQ in children GI Sx, Oral Lead Line, Interfility, Delayed Puberity in Girls |
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Low IQ, Hearing Loss in Children
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Consequences of Lead Exposure
30 year halflife, stored in bones and teeth inhibits enzymes which incorporate iron into heme: ferrochelatase & delta aminolevulinic acid dehydratase Competes with Ca2+ interferes with normal remodelling of epiphysial cartilage in children inhibits healing of fractures Impaired 1, 25 DH vitamin D metabolism Inhibits membrane-associated enzymes coarse basophilic stippling, hemolysisis renal damage-->htn, gout demyelination: Nerve transmission deficits-->wrist drop, ankle drop brain dvlpt deficits--> Low IQ in children |
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hypochromic microcitic anemia with coarse basophilic stippling in RBCs
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30 year halflife, radiodense desposits in bones and teeth
blocks iron into heme: ferrochelatase & delta aminolevulinic acid dehydratase hypochromic microcytic Anemia w/ basophilic stippling Competes with Ca2+, Impaired 1, 25 DH vitamin D metabolism interferes with normal remodelling of epiphysial cartilage in children inhibits healing of fractures Inhibits membrane-associated enzymes hemolysisis renal damage-->htn, gout demyelination:-->wrist drop, ankle drop,--> Low IQ in children GI Sx, Oral Lead Line, Interfility, Delayed Puberity in Girls |
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Labs for Iron Deficiency Anemia
Morphology Serum Iron TIBC Transferrin % Saturation Ferritin Other Findings |
Morphology: Microcytic
Serum Iron: Decreased TIBC Transferrin: Increased % Saturation: Decreased Ferritin: Decreased Other Findings: Decreased Marrow Iron |
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Labs for Chronic Disease Anemia
Morphology Serum Iron TIBC Transferrin % Saturation Ferritin Other Findings |
Morphology: Microcytic
Serum Iron:Decreased TIBC Transferrin: Decreased % Saturation: Decreased Ferritin: Increased Other Findings: Increased Marrow Iron |
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Labs for Beta Thalassemia
Morphology Serum Iron TIBC Transferrin % Saturation Ferritin Other Findings |
Morphology: Microcytic
Serum Iron: Normal TIBC Transferrin: Normal % Saturation: Normal to Increased Ferritin: Normal to Increased Other Findings: Abnormal Hemoglobin Electrophoresis |
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Labs for Alpha Thalassemia
Morphology Serum Iron TIBC Transferrin % Saturation Ferritin Other Findings |
Morphology: Microcytic
Serum Iron: Normal TIBC Transferrin: Normal % Saturation: Normal to Increased Ferritin: Normal to Increased Other Findings: Abnormal Hemoglobin Electrophoresis |
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Labs for Sideroblastic Anemia
Morphology Serum Iron TIBC Transferrin % Saturation Ferritin Other Findings |
As seen in lead poisoning
Morphology: Microcytic Serum Iron: Increased TIBC Transferrin: Normal to Decraeased % Saturation: Incresed Ferritin: Incresed Other Findings: Ringer Sideroblasts, Basophilic st |
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Morphology: Microcytic
Serum Iron: Decreased TIBC Transferrin: Increased % Saturation: Decreased Ferritin: Decreased |
Labs for Iron Deficiency Anemia
Other Findings: Decreased Marrow Iron |
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Morphology: Microcytic
Serum Iron:Decreased TIBC Transferrin: Decreased % Saturation: Decreased Ferritin: Increased |
Labs for Chronic Disease Anemia
Other Findings: Increased Marrow Iron |
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Morphology: Microcytic
Serum Iron: Normal TIBC Transferrin: Normal % Saturation: Normal to Increased Ferritin: Normal to Increased Other Findings: Abnormal Hemoglobin Electrophoresis |
Labs for Thalassemias: Alpha or beta
Other Findings: Abnormal Hemoglobin Electrophoresis |
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Morphology: Microcytic
Serum Iron: Increased TIBC Transferrin: Normal to Decraeased % Saturation: Incresed Ferritin: Incresed |
Labs for Sideroblastic Anemia, as seen in lead poisoning
Other Findings: Ringer Sideroblasts, Basophilic stippling |
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Dark Line on Gingiva
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Consequences of Lead Exposure
30 year halflife, radiodense desposits in bones and teeth blocks iron into heme: ferrochelatase & delta aminolevulinic acid dehydratase hypochromic microcytic Anemia w/ basophilic stippling Competes with Ca2+, Impaired 1, 25 DH vitamin D metabolism interferes with normal remodelling of epiphysial cartilage in children inhibits healing of fractures Inhibits membrane-associated enzymes hemolysisis renal damage-->htn, gout demyelination:-->wrist drop, ankle drop,--> Low IQ in children GI Sx, Oral Lead Line, Interfility, Delayed Puberity in Girls |
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Langerhans Cell Histiocytoses
Morphology, EM Molecular Features Specturm, features of Acute Dissemination |
EM: Birbeck Granules + CD1a
spectrum from malignant to benign "eosinophilic granulomas" lease severre: DCs sends out cytokines recruit eos extreme form Letterer-Siwe Diease = Multisystem, rapidly fatal |
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Letterer-Siwe Diease
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Multisysstem Langerhans Cell Histiocytoses
EM: Birbeck Granules + CD1a Rash, hepatosplenomegaly & Lymphadenopathy, Osteolysis in Bones, Anemia thrombocytopenia w/ infx, Lung invovlement Rapidly fatal, responds well to chemo |
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Hand-Schuller Christian Triad
not on exam |
Eosinphilic Granulomas of Calvarial Bone (Skull)
Exopthalmos Diabetes insipidus from pituitary invovlement Possible Manifestations of Langerhans Histiocytosis Birbeck Granules + CD1a |
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Eosinphilic Granulomas of Calvarial Bone
Exopthalmos Diabetes insipidus not on exam |
Hand-Schuller Christian Triad
Possible Manifestations of Langerhans Histiocytosis Birbeck Granules + CD1a |
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Eosinophilic Granuloma
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Least severe manifestation of langerhans histiocytosis
CD1a + Birbeck granules Multicellular granuloma with langerhans cells and eos being most prominemt Comonly in hematopoietic bones Good Prognosis |
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Bone Pain, Hypercalcemia, Recurrent Infxn, Amyloidosis, Hyperviscosity
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Features of Multiple Myeloma
50 yo Blacks w/ genetic predisposition & chronic inflammation (HIV, RA, osteopmyelitis) IL6 released as autocrine signal, also drives osteoclasts-->osteolysis Excess Ig--> hyperviscosity, renal damage, suppress humoral immunity -->infxn Skeletal defects "punched out bones" w/ pain and hypercalemia Myeloma Kidney: Bence Jones light chain precipitates forming Casts Multinucleate plasma Cells w/ Russel Boddies Ig Cytoplasmic Inclusions 10% systemic amyloidosis congo red leukemia is late sign Labs: M protein on electrophoresis, Rouleaux Formation, Bence-Jones in Urine |
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Plasma Cell Dyscraisas
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Neoplasms of Terminally Differentiated B Cells
Leads to monoclonal gammopathy May produce M (myeloma) protein--not IgM Complement L (Light) Chains--may cause amyloidosis H (heavy) Chains Most common is MGUS- monoclonal gammopathy of undetermined significance Rouleaux Formation |
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Rouleaux Formation
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Strings of RBC's
Albumin has a negative charge RBC's have an negative charge globulins have a positive charge indicative of increased globulin/reduced albumin indicative of plasma cell dyscrasia (neoplasm of terminally differentiated--multiple myeloma, MGUS, Waldenstrom's macroglobulinemia, Heavy chain disease, Light chain amyloidosis) |
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Multiple Myeloma
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50 yo Blacks w/ genetic predisposition & chronic inflammation (HIV, RA, osteopmyelitis)
Bone Pain, Hypercalcemia, Recurrent Infxn, Amyloidosis, Hyperviscosity Excess Ig--> hyperviscosity, renal damage, suppress humoral immunity -->infxn IL6 released as autocrine signal, also drives osteoclasts-->osteolysis Skeletal defects "punched out bones" w/ pain and hypercalemia Myeloma Kidney: Bence Jones light chain precipitates forming Casts Multinucleate plasma Cells w/ Russel Boddies Ig Cytoplasmic Inclusions 10% systemic amyloidosis congo red leukemia is late sign Labs: M protein on electrophoresis, Rouleaux Formation, Bence-Jones in Urine |
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Most Common Plasma Cell Dyscrasia
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MGUS
Monoclonal gammopathy of undetermined significance |
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Bence Jones Protein
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Multiple Myeloma: 50 yo Blacks w/ genetic predisposition & chronic inflammation (HIV, RA, osteopmyelitis)
Bone Pain, Hypercalcemia, Recurrent Infxn, Amyloidosis, Hyperviscosity Excess Ig--> hyperviscosity, renal damage, suppress humoral immunity -->infxn IL6 released as autocrine signal, also drives osteoclasts-->osteolysis Skeletal defects "punched out bones" w/ pain and hypercalemia Myeloma Kidney: Bence Jones light chain precipitates forming Casts Multinucleate plasma Cells w/ Russel Boddies Ig Cytoplasmic Inclusions 10% systemic amyloidosis congo red leukemia is late sign Labs: M protein on electrophoresis, Rouleaux Formation, Bence-Jones in Urine Dx: CRAB: hypercalcemia, renal insufficiency, anemia, bone lesions |
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CRAB
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hypercalcemia, renal insufficiency, anemia, bone lesions
Multiple Myeloma: 50 yo Blacks w/ genetic predisposition & chronic inflammation (HIV, RA, osteopmyelitis) Bone Pain, Hypercalcemia, Recurrent Infxn, Amyloidosis, Hyperviscosity Excess Ig--> hyperviscosity, renal damage, suppress humoral immunity -->infxn IL6 released as autocrine signal, also drives osteoclasts-->osteolysis Skeletal defects "punched out bones" w/ pain and hypercalemia Myeloma Kidney: Bence Jones light chain precipitates forming Casts Multinucleate plasma Cells w/ Russel Boddies Ig Cytoplasmic Inclusions 10% systemic amyloidosis congo red leukemia is late sign Labs: M protein on electrophoresis, Rouleaux Formation, Bence-Jones in Urine |
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Solitary plasmacytoma
Not on test |
localized tumor of plasma cells,
M componenet minimal or absent in bone marrow preceeds dvlpt of multiple myeloma by 10+ years URT, Lungs curable by surgery |
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MGUS
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aSx M protein
dx of exclusion most common monoclonal gammopathy precancer, dvlpt correlates with [M] |
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Russell Bodies
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Ig cytoplasmic inclusions found in Multiple Myeloma: 50 yo Blacks w/ genetic predisposition & chronic inflammation (HIV, RA, osteopmyelitis)
Bone Pain, Hypercalcemia, Recurrent Infxn, Amyloidosis, Hyperviscosity Excess Ig--> hyperviscosity, renal damage, suppress humoral immunity -->infxn IL6 released as autocrine signal, also drives osteoclasts-->osteolysis Skeletal defects "punched out bones" w/ pain and hypercalemia Myeloma Kidney: Bence Jones light chain precipitates forming Casts Multinucleate plasma Cells w/ Russel Boddies Ig Cytoplasmic Inclusions 10% systemic amyloidosis congo red leukemia is late sign Labs: M protein on electrophoresis, Rouleaux Formation, Bence-Jones in Urine Dx: CRAB: hypercalcemia, renal insufficiency, anemia, bone lesions |
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Monoclonal IgM
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Lymphoplasmacytic Lymphoma
Hyperviscosity Syndrome, Waldenstorms Macroglobulinemia Involves LN's cold agglutinins Bleeding, Cryoglobulinemia, Raynaud's No punched out bone lesions neuologic sx Review more if time |
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Fnx of the spleen
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Blood Filtration:
Senescent RBC's Antibody Bound RBC's Howell Jolly & Heinz Body Removal Removes bacteria, macromolecules, cell debris 2ndry organ with humoral immunity fnx Source ofLymphoreticular cells Extramedullary Hematopoiesis Rserve for RBC's & 1/3 of platelets |
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What can cause splenomegaly
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Ifnxn, Congestion, Lymphoehematogenous Disoderrs,
Immunologic inflmmatory conditions: RA SLE Glycogen storage diseases & Gauchers disease Infx of the spleen (Mono, CMV) Sx: early sateity hyperspenism: 1splenomegaly 2 redxn in any blood cell line 3 compensatory bone marrow hyperplasia |
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What cancer's involve the spleen?
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malignant melanoma
lung cancer lymphohematopoietic malignancy |
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Rupture of spleen
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Trauma or rupture
normal spleen never ruptures spontaneously -Mono, Malaaria, Tycohid, Acute Splenitis, Leukemia Massive intraabdominal hemoorhage, hypovolemic shock requires surgery afterward: CBC: NRBC's Howell Jolly Bodies, target cells vulnerable to encapsulated infxn, S pneumoniae |
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Spleen coated with fibrin
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perisplenitis
nonspecific actue splenitis 2ndry to bloodborne infxn |
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perisplenitis
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nonspecific actue splenitis 2ndry to bloodborne infxn
Spleen coated with fibrin |
