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HemeOnc Flashcards

1
Q

Stain for erythrocytes

A

Blue on Wright-Giemsa stain - Stains residual reticular rRNA

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2
Q

GpIb and GpIIb/IIIa

A 
GpIb = vWF receptor
GpIIb/IIIa = Fibrinogen receptor
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3
Q

Platelet dense granules and a-granules

A 
Dense = ADP, Ca
a-granules = vWF, Fibrinogen, Fibronectin
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4
Q

Neutrophil Specific and Azurophilic granules

A

Specific = LAP, Collagenase, Lysozyme, Lactoferrin

Azurophilic = Proteinases, Acid phosphatase, Myeloperoxidase, b-Glucuronidase

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5
Q

Neutrophilic chemotactic agents

A 
C5a
IL-8
LTB4
Platelet activating factor
Kallikrein
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6
Q

Hypersegmented neutrophils (6+ lobes)

A

Vit B12/Folate deficiency

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7
Q

Kidney shaped nucleus

“Frosted glass” cytoplasm

A

MONOCYTE

Becomes macrophage in tissues

Note - Lipid A from LPS binds CD14 on macrophages and leads to septic shock

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8
Q

Produced by eosinophils

A

Major basic protein (helminthotoxin)

Histaminase

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9
Q

Causes of eosinophilia (5)

A 
Neoplasia
Asthma
Allergy
Chronic adrenal insufficiency
Parasites
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10
Q

Produced by basophils

A

Heparin
Histamine
Leukotrienes

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11
Q

Drug preventing mast cell degranulation

A

Cromolyn sodium

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12
Q

Lymphocytes with eccentric nuclei and large eccentric nucleus

A

PLASMA CELL

Normally do not circulate

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13
Q

Sites and timeline of fetal erythropoiesis

A

Yolk sac (3 - 8 weeks)
Liver (6 weeks - Birth)
Spleen (10 - 28 weeks)
Bone marrow (18 weeks - Adult)

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14
Q

Fetal to adult hemoglobin development

A

HbF (a2y2) has y replaced by b to become HbA1 (a2b2)

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15
Q

Universal donor and acceptor of plasma

A

Universal donor - AB (no IgMs)
Universal acceptor - O (both IgGs)

Note - Opposite of universal donor and acceptor of RBCs

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16
Q

Mechanism of Rh hemolytic disease of the newborn

A

Rh- mother has Rh+ pregnancy
Exposure to Rh+ blood results in anti-D IgG
Subsequent Rh+ pregnancies anti-D IgG crosses placenta

Treat with RhoGAM (anti-D IgG) during first pregnancy to opsonize Rh+ fetal erythrocytes

Note - Resulting Coombs+ anemia and hydrops fetalis is due to maternal antibodies as the infant is not yet making its own antibodies

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17
Q

Mechanism of ABO hemolytic disease of newborn - mild jaundice resolved with phototherapy or exchange transfusion and also see hyperbilirubinemia

immune hemolytic anemia due to ABO incompatibility. Infant A or B and mother is O.

A

Blood type O mother with A, B, or AB fetus makes anti-A or anti-B IgG which crosses placenta - Does not worsen with future pregnancies

Note - Type O mothers make IgG instead of IgM

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18
Q

Order of hemoglobin migration (greatest to least) on gel electrophoresis - Negatively charged so moves from Cathode (-) to Anode (+)

(“A Fat Santa Claus”)

A

HbA
HbF
HbS
HbC

Note - Replacement of Glutamic acid (-) with Valine (neutral) in HbS and Lysine (+) in HbC responsible for relative migration on gel

Note - Paradoxically, HbF has a less positive binding pocket for 2,3-BPG compared to HbA

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19
Q

Effects of Bradykinin - Converted from HMWK by Kallikrein

A

Vasodilation
Permeability
Pain

Note - HMWK also helps activate XII

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20
Q

Contact activation (intrinsic) pathway

A

Collagen, basement membrane, activated platelets exposed

XII = XIIa
XIIa + XI = XIa
XIa + IX = IXa
IXa + VIIIa + X + Ca + Phospholipid = Xa

Note - vWF carries and protects VIII

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21
Q

Tissue factor (extrinsic) pathway

A

VII + TF + Ca + Phospholipid = VIIa

VIIa + X + Ca + Phospholipid = Xa

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22
Q

Combined pathway

A

Xa + Va + II (Prothrombin) + Ca + Phospholipid = IIa
IIa (Thrombin) + I (Fibrinogen) = Ia
Ia (Fibrin) + XIIIa + Ca = Fibrin mesh

Note - IIa (Thrombin) activates V, VIII, and XIII

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23
Q

Fibrinolytic system

A

Plasminogen + tPA = Plasmin
Plasmin + Fibrin mesh = Fibrin degradation products

Note - Also destroys coagulation factors

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24
Q

Mechanism of Vit K procoagulation

Defect results in…
Normal BT
Increased PT
Increased PTT

A

Oxidized Vit K reduced by Epoxide Reductase
Reduced Vit K cofactor for y-Glutamyltransferase
y-Glutamyl Transferase activates X, IX, VII, II, C, S

Note - Neonates lack enteric bacteria which produce Vit K and can develop ICH (downset eyes, bulging fontanelle)

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25
Anticoagulation cascade
Protein C + Thrombin-Thrombomodulin complex Activated Protein C Activated Protein C + Protein S cleaves Va, VIIIa Note - Factor V Leiden is protein C resistant Va
26
Principal target of AT
II (Thrombin) Xa Note - Also X, IX, VII, II, XII, XI
27
Mechanism of platelet plug formation - primary hemostasis Note = Unstable/easily dislodged plug stops bleeding until secondary hemostasis (coagulation cascade)
Endothelial damage releases Endothelin (transient vasoconstriction) vWF binds to exposed collagen Platelet Gp1b bind vWF at site of injury (specific), then undergo conformational change Platelets release ADP and Ca ADP helps adherence platelets to endothelium and induces GpIIb/IIIa expression on platelets Fibrinogen binds GpIIb/IIIa and links platelets Note - Decreased blood flow and TXA2 (platelets) cause further aggregation, while increased blood flow and PGI2/NO (endothelial cells) inhibit aggregation
28
Source of vWF
Weibel-Palade bodies of endothelial cells | a-granules of platelets
29
Coagulation factors produced by endothelial cells
``` vWF VIII Thromboplastin (II to IIa) tPA PGI2 ``` Note - Also express thrombomodulin membrane protein for activation of Protein C
30
Acanthocyte (spur cell) - spiny surface
Liver disease | Abetalipoproteinemia (B-48, B-100)
31
Basophilic stippling
Lead poisoning Sideroblastic anemia Myelodysplastic syndromes
32
Dacrocyte (tear drop cells)
Bone marrow infiltration (e.g. myelofibrosis) Note - From being mechanically squeezed out of the bone marrow
33
Degmacyte (bite cells)
G6PD deficiency
34
Echinocyte (burr cell) Note - More uniform, smaller projections than Acanthocyte
``` ESRD Liver disease Pyruvate kinase deficiency Microangiopathic hemolytic anemia Macroangiopathic hemolytic anemia ```
35
Elliptocyte
Hereditary elliptocytosis Note - Mutation in Spectrin
36
Macro-ovalocyte
``` Megaloblastic anemia (+ hypersegmented PMNs) Marrow failure ```
37
Ringed sideroblasts - Stain with Prussian blue due to iron-laden mitochondria surrounding nucleus Note - Nucleated RBC
Sideroblastic anemia Note - Only present on marrow staining
38
Schistocyte - Fragments of RBCs Note - Includes helmet cell
``` Microangiopathic hemolytic anemias... DIC TTP/HUS SLE Malignant HTN ``` Macroangiopathic hemolytic anemias... Aortic stenosis Prosthetic valves
39
Causes of sickling
Dehydration Deoxygenation High altitude
40
Spherocytes
Hereditary spherocytosis | Drug/Infection induced hemolytic anemia
41
Target cell
HbC disease Asplenia Liver disease Thalassemia
42
Heinz bodies - Peripheral inclusions of oxidized and denatured hemoglobin
G6PD deficiency hemolysis occurs following exposure to oxidants or infections Oxidation of Hb SH to SS bonds allows precipitation and subsequent phagocytic damage (e.g. bite cells)
43
Howell-Jolly bodies - basophilic nuclear remnants normally removed by splenic macrophages
Asplenia
44
Microcytic anemias
``` Iron deficiency a-thalassemia b-thalassemia Lead poisoning Sideroblastic anemia ```
45
Fatigue Conjunctival pallor Pica Spoon nails Decreased Iron Decreased Ferritin Increased TIBC/Transferrin Decreased Transferrin Saturation (Fe/TIBC)
IRON DEFICIENCY ANEMIA Microcytosis with hypochromia Note - Increased TIBC and Decreased Transferrin Saturation seen in OCP use but Fe levels are normal
46
Various presentations of a-thalassemia
4 allele deletion - Hb Barts (y4) with hydrops fetalis 3 allele deletion - HbH disease (b4) 2 allele deletion - Mild clinical anemia 1 allele deletion - Clinically silent Note - Trans deletions (opposite chromosomes) more common in African Americans
47
``` Target cells Anisopoikilocytosis Increased HbF Increased HbA2 (a2d2) Hepatosplenomegaly (extramedullary hematopoiesis) Crew cut on skull XR/Chipmunk facies Secondary hemochromatosis (transfusions) B19 aplastic crisis ``` Note - Becomes symptomatic as HbF declines (6 months)
B-THALASSEMIA MAJOR Point mutation in splice sites and promoter sequences leads to absent b chain Microcytic, hypochromic anemia Note - HbA1c readings artificially high due to HbA1 underproduction
48
Diagnosis of b-thalassemia minor - underproduction rather than absence of b globulin
HbA2 (a2d2) > 3.5% - usually clinically silent
49
``` Mechanism of... Burton lines on long bone metaphyses and gingiva Encephalopathy Abdominal colic Wrist and foot drop Microcytic anemia with normal Fe studies ```
LEAD POISONING Inhibited heme synthesis due to deficient... d-ALA dehydratase (cytoplasmic) Ferrochelatase (mitochondrial) Increased RBC d-ALA or Protoporphyrin Inhibition of rRNA degradation leading to aggregate formation - Basophilic stippling (peripheral) and ringed sideroblasts (marrow) Treat with... Dimercaprol/EDTA Succimer chelation in children
50
``` Increased Iron Increased Ferritin Normal/Decreased TIBC Ringed sideroblasts in marrow Basophilic stippling in peripheral smear ```
SIDEROBLASTIC ANEMIA X-linked d-ALA Synthase (mitochondrial) gene mutation results in defective heme synthesis ``` Non-genetic causes include... Vit B6 deficiency (Isoniazid) Copper deficiency Lead Alcohol ``` Treat with... Pyridoxine - Cofactor for d-ALA synthase Note - Converts Glycine, Succinyl-CoA, and B6 to d-ALA
51
Macrocytic anemias Note - Megaloblastic (delayed DNA synthesis) also presents with Hypersegmented neutrophils and Glossitis
``` Megaloblastic (> 115): Folate deficiency B12 deficiency Orotic aciduria NO (inhibits Methionine Synthase) ``` ``` Non-megaloblastic: Liver disease Alcoholism Hypothyroidism Diamond-Blackfan anemia Myelodysplastic syndromes ``` Note - Increased cell turnover (e.g. SCD) causes folate deficiency
52
``` Rapid-onset anemia within first year Short stature Craniofacial abnormalities Triphalangeal thumbs Increased HbF Normal total Hb ```
DIAMOND-BLACKFAN ANEMIA Intrinsic defect in erythroid progenitor cells
53
``` Indirect hyperbilirubinemia Decreased haptoglobin Increased LDH Hemoglobinuria Hemosiderinuria Urobilinogen Schistocytes, Reticulocytes on peripheral smear ```
INTRAVASCULAR HEMOLYSIS Mechanical hemolysis Paroxysmal nocturnal hemoglobinuria Microangiopathic hemolytic anemia
54
``` Indirect hyperbilirubinemia Increased LDH Urobilinogen No hemoglobinuria No hemosiderinuria Spherocytes on peripheral smear ```
EXTRAVASCULAR HEMOLYSIS
55
Normocytic, nonhemolytic anemia Decreased Iron Increased Ferritin Decreased TIBC/Transferrin Note - Normocytic, nonhemolytic anemias have normal or decreased Retic counts
ANEMIA OF CHRONIC DISEASE Increased Hepcidin from liver binds Ferroportin on intestinal mucosa and macrophages decreasing Iron release from macrophages and absorption from the gut Treat with EPO
56
``` Fatigue pallor Purpura Mucosal bleeding Petechiae Infection ``` ``` Severe normocytic, nonhemolytic anemia Leukopenia Thrombocytopenia Decreased reticulocyte count Increased EPO Normal cell morphology Hypocellular bone marrow with fatty infiltration ```
APLASTIC ANEMIA ``` Caused by... Radiation Drugs (Chloramphenicol, Sulfonamide, Carbamazepine) Toxins (Benzene, Solvents) Viral (B19, EBV, HIV) Fanconi anemia Autoimmune ``` ``` Treat with... Immunosuppression Transfusion GM-CSF Bone marrow allograft ```
57
``` Aplastic anemia Short stature Cafe-au-lait spots Thumb and Radius defects Increased incidence of cancer ```
FANCONI ANEMIA DNA cross-link repair defect
58
``` Extravascular hemolysis (e.g. viral illness) Aplastic crisis (B19) Splenomegaly Osmotic fragility+ Elevated MCHC ```
HEREDITARY SPHEROCYTOSIS Autosomal dominant defect in proteins interacting with RBC membrane (e.g. Spectrin, Ankyrin) - premature removal by spleen Treat with splenectomy
59
``` Episodic... Extravascular and intravascular hemolysis Back pain Hemoglobinuria Heinz bodies Bite cells ```
G6PD DEFICIENCY X-linked recessive defect in G6PD - Prevents reduction of NADP to NADPH which is used to reduce Glutathione
60
Extravascular hemolysis in a newborn Splenomegaly (red pulp hyperplasia) Echinocytes
PYRUVATE KINASE DEFICIENCY Autosomal recessive mutation resulting in decreased ATP leading to rigid RBCs Note - No mitochondria in RBC so all energy must come from lactate
61
Splenomegaly Extravascular hemolysis Hemoglobin crystals in RBCs Target cells
HBC DISEASE Glutamic acid to Lysine mutation in b-globulin Note - HbSC milder disease than HbSS
62
``` Coombs- intravascular hemolysis Pancytopenia Venous thrombosis CD55/59- RBCs Increased incidence of leukemia ```
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA Impaired synthesis of GPI anchor for decay-accelerating factor - Deficient protection of RBC membrane from complement lysis Treat with Eculizumab (terminal complement inhibitor)
63
``` Intravascular and extravascular hemolysis Crew-cut on skull XR (marrow expansion) Possibly Howell-Jolly bodies Absent HbA Increased HbF ```
SICKLE CELL ANEMIA Glutamic acid to Valine mutation results in polymerization of deoxygenated HbS Treat with... Hydroxyurea (increases HbF) Hydration Note - Newborns initially asymptomatic due to high HbF
64
Complications of SCD
``` Aplastic crisis (B19) Splenic sequestration/congestion (infancy) Splenic atrophy/fibrosis (childhood) Encapsulated infections Renal papillary necrosis Medullary infarcts (microhematuria) Pigmented gallstones Vaso-occlusive crises (Dactylitis, Priapism, ACS, Avascular necrosis, Stroke) ```
65
Warm autoimmune hemolytic anemia
Coombs+ IgG mediated... SLE CLL a-Methyldopa
66
Cold autoimmune hemolytic anemia
Coombs+ IgM and Complement mediated... CLL Mycoplasma EBV (Mononucleosis) Note - May cause painful blue fingers and toes with cold exposure
67
Normocytic anemias
Non-hemolytic: ACD CKD Aplastic anemia Hemolytic: Intrinsic - Hereditary spherocytosis, G6PD, Pyruvate Kinase, HbC, Paroxysmal Nocturnal Hemoglobinuria, SCD Extrinsic - AIHD, Micro/Macroangiopathic, Infections
68
Increased Iron Increased Ferritin Decreased TIBC/Transferrin Increased Transferrin Saturation
Hemochromatosis
69
Hematologic effects of Corticosteroids
Neutrophilic - Decreased adhesion and extravasation Leukopenia - Increased apoptosis Eosinopenia - Sequestration in lymph nodes
70
Left shift | Immature RBCs
LEUKOERYTHROBLASTIC REACTION Severe anemia Marrow fibrosis Tumor taking up space in marrow
71
Painful abdomen Port-wine colored urine Polyneuropathy Psychological disturbances Precipitated by... Drugs (Cytochrome P-450 inducers) Alcohol Starvation
ACUTE INTERMITTENT PORPHYRIA Porphobilinogen Deaminase (cytoplasmic) deficiency results in accumulation of... d-ALA Porphobilinogen Treat by inhibiting ALA synthase with Glucose/Heme Note - Also paradoxically Coproporphyrinogen (urine)
72
Blistering cutaneous photosensitivity | Tea-colored urine
PORPHYRIA CUTANEA TARDA Uroporphyrinogen Decarboxylase (cytoplasmic) deficiency results in accumulation of Uroporphyrinogen (tea-colored urine)
73
``` Child with... Nausea Vomiting Gastric bleeding Lethargy Opacity in stomach on CXR ``` Note - Long term may result in scarring and GI obstruction
IRON POISONING Cell death due to peroxidation of membrane lipids Treat with... IV Deferoxamine Oral Deferasirox Dialysis
74
Factors tested by PT Note - INR is normalized PT
Extrinsic and Common pathways... VII, X, V, II, I Note - Follow PT/INR with Warfarin use
75
Factors tested by PTT
Intrinsic and Common pathways... | All except VII and XIII
76
Deficiencies and treatment of... Hemophilia A Hemophilia B Hemophilia C Note - All present with isolated elevated aPTT
VIII (X-linked recessive) IX (X-linked recessive) XI (Autosomal recessive) All receive Desmopressin and appropriate factor
77
``` Microhemorrhage... Bleeding mucous membranes Epistaxis Petechiae Purpura Increased BT Decreased PC ```
Platelet disorders ``` Includes... Bernard-Soulier Glanzmann thrombasthenia HUS ITP TTP ```
78
Defect in platelet plug formation - large platelets
BERNARD-SOULIER SYNDROME autosomal recessive - deficient GpIb leads to impaired platelet-vWF adhesion (GpIb normally bind vWF) and also diminished production of platelets by megakaryocytes in bone marrow presents with petechia, purpura, menorrhagia, and prolonged bleeding with trauma and surgery decreased platelet count, presence of giant platelets, prolonged bleeding time, and normal PT and PTT abnormal platelet aggregation with ristocetin
79
Defect in platelet plug formation - no platelet clumping on smear Note - Platelet count usually normal
GLANZMANN THROMBASTHENIA Deficient GpIIb/IIIa leads to impaired platelet-platelet aggregation
80
``` Child with... Diarrhea (bloody) Thrombocytopenia Microangiopathic hemolytic anemia with schistocytes Acute renal failure ``` ``` decreased hemoglobin decreased haptoglobin increased lactate dehydrogenase increased unconjugated bilirubin increased bleeding time ```
HEMOLYTIC UREMIC SYNDROME (TTP-HUS) Shiga toxin-producing EHEC (O157:H7) leads to microthrombi formation or shigella dysenteriae Treat with Plasmapheresis Note - No EHEC or diarrhea in adults
81
Viral illness followed by... Petechiae Mucosal bleeding Increased megakaryocytes/hyperplasia on bone marrow smear Increased BT Normal PT Normal aPTT Decreased PC
IMMUNE THROMBOCYTOPENIA (ITP) antibodies against platelet glycoproteins which results in platelet removal by splenic reticuloendothelial system Anti-GpIIb/IIIa antibodies leads to splenic macrophage consumption of platelet-antibody complex MUST rule out secondary thrombocytopenia due to lupus, viral infection, drugs etc. acute form in children chronic form in adults Treat with... Steroids IVIG Splenectomy if refractory
82
``` Fever Thrombocytopenia Microangiopathic hemolytic anemia Neurologic symptoms Renal symptoms ``` Schistocytes Increased LDH Normal PT Normal aPTT
THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) Deficiency of ADAMTS13 (vWF metalloprotease) leads to decreased degradation of vWF multimers - Increased platelet adhesion, aggregation, and thrombosis Treat with... Steroids Plasmapheresis
83
``` Increased BT Normal PT Normal/Increased aPTT Normal platelet count No aggregation with ristocetin cofactor assay ``` Note - PTT may be normal
VON WILLEBRAND DISEASE Autosomal dominant deficiency in vWF leads to elevated PTT (carries/protects VIII), and defect in platelet plug formation (GpIb-vWF adhesion) Treat with Desmopressin - Releases vWF from endothelial cells note:vWf is produced by endothelial cells and megakaryocytes
84
``` Increased BT Increased PT Increased PTT Decreased platelets/Thrombocytopenia Decreased Fibrinogen Increased D-dimer (fibrin degradation product) Decreased V, VIII Schistocytes ```
DISSEMINATED INTRAVASCULAR COAGULATION Is due to massive tissue factor(thromboplastin) release which generates thrombin and initiates the clotting cascade ``` Caused by... Gram- sepsis Trauma Obstetric complications Acute pancreatitis Malignancy Nephrotic syndrome Transfusion ```
85
Diminished increase in PTT with Heparin administration
ANTITHROMBIN DEFICIENCY May be inherited or acquired (renal failure) - leads to decreased inhibition of IIa and Xa
86
Skin necrosis with hemorrhage after administration of Warfarin
PROTEIN C/S DEFICIENCY
87
Prothrombin gene mutation
Mutation in 3' UTR - Increased production and venous clots
88
Mechanism of hypocalcemia following transfusion
Citrate in packed RBCs is a Ca chelator
89
Ingredients of Cryoprecipitate
``` vWF VIII XIII Fibrinogen Fibronectin ```
90
Used to treat... DIC Cirrhosis Immediate Warfarin reversal
FRESH FROZEN PLASMA
91
B cell neoplasm with... Multiple lymph node involvement Noncontiguous spread Extranodal involvement Note - May be associated with HIV or autoimmune disease
NON-HODGKINS LYMPHOMA
92
``` Young adult or >55 y/o (bimodal) with... Low-grade fever Night sweats Weight loss Localized, single group of nodes "Owl Eyes" cells ``` Note - Associated with EBV
HODGKIN'S LYMPHOMA Contiguous spread Relatively good prognosis
93
Surface markers for "Owl Eyes" Note - Necessary but not sufficient for diagnosis of Hodgkin's lymphoma
REED-STERNBERG CELLS B-cell origin CD15 CD30
94
Non-Hodgkin's lymphoma... Associated with EBV Jaw lesions (Africa) or pelvic/abdominal lesions "Starry sky" - Sheet of lymphocytes with interspersed "tingible body" macrophages
BURKITT LYMPHOMA t(8;14) - Translocation of c-myc and heavy-chain Ig leads to activation of c-myc Note - Heavy-chain Ig constitutively expressed so translocation near this gene causes overexpression
95
Most common type of non-Hodgkin's lymphoma in adults
DIFFUSE LARGE B-CELL LYMPHOMA
96
Non-Hodgkin's lymphoma with... Painless waxing and waning lymphadenopathy Small cleaved cells, large cells, or mixture
FOLLICULAR LYMPHOMA t(14;18) - Translocation of heavy-chain IgG and Bcl-2 Note - Bcl-2 inhibits apoptosis and so this translocation promotes cell proliferation
97
Aggressive non-Hodgkin's' lymphoma presenting in adult males
MANTLE CELL LYMPHOMA t(11;14) - Translocation of cyclin D1/bcl-1 (G1 to S) and heavy-chain IgG also seen in multiple myeloma
98
``` Non-Hodgkin's lymphoma with... Confusion Memory loss Seizures Mass lesions on MRI ```
PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA Considered an AIDS defining illness
99
``` Non-Hodgkin's lymphoma with... T-cell origin Cutaneous lesions Lytic bone lesions Hypercalcemia ``` Note - Associated with IVDU
ADULT T-CELL LYMPHOMA Caused by HTLV
100
Non-Hodgkin's lymphoma with... T-cell origin Skin patches and plaques CD4+ cells with "cerebriform" nuclei
MYCOSIS FUNGOIDES (CUTANEOUS T CELL LYMPHOMA) May progress to Sezary syndrome (T cell leukemia)
101
- Hypercalcemia - Bone pain - Back pain - Lytic bone lesions leading to pathologic fractures - Elevated ESR - Serum monoclonal spike - Renal involvement (Bence Jones Ig light chains) - Increased infections - Primary amyloidosis - Anemia Rouleaux RBC formation "Fried egg" plasma cells Intracytoplasmic inclusions (Ig) M spike (y) on serum protein electrophoresis
MULTIPLE MYELOMA Monoclonal plasma cell cancer in marrow -that produce excess amounts of immunoglobulin (usually IgG or IgA) --> M spike Excess free kappa and lambda chains may be filtered into urine (bence jones proteins) Note - Isolated M spike without CRAB symptoms is MGUS
102
Cause of hyperviscosity syndrome... Blurred vision Raynaud phenomenon
WALDENSTROM MACROGLOBULINEMIA M spike of IgM - no CRAB findings
103
``` Defects in cell maturation of all nonlymphoid lineages... Bleeding (thrombocytopenia) Fatigue (anemia) Recurrent infections (myelocytes) Also decreased mast cells ``` Ringed sideroblasts on marrow smear Note - Myelocytes include basophils, eosinophils, neutrophils, macrophages
MYELODYSPLASTIC SYNDROMES ``` Caused by... De novo mutations Radiation Chemotherapy Benzene ``` Note - May transform to AML
104
Neutrophils with bilobed nuclei - typically seen after chemotherapy
PSEUDO-PELGER-HUET ANOMALY
105
``` Child with... Anemia Thrombocytopenia Neutropenia Increased blasts on peripheral smear TdT+ (pre-T, pre-B) Mostly CD10+ (pre-B) ``` Note - Associated with Down's syndrome
ACUTE LYMPHOBLASTIC LEUKEMIA/LYMPHOMA (ALL) t(12;21) has a better prognosis Spreads to Testes and CNS T-Cell ALL may present as mediastinal mass - SVC syndrome, dysphagia, dyspnea/stridor
106
Elderly with... Autoimmune hemolytic anemia Smudge cells on peripheral smear CD19+ CD20+ (B-cell) with CD5+
CHRONIC LYMPHOCYTIC LEUKEMIA (SMALL LYMPHOCYTIC LEUKEMIA) Treat with Alemtuzumab or Rituximab
107
CLL/SLL transformation into aggressive lymphoma - most commonly DLBCL
RICHTER TRANSFORMATION
108
``` Adult male with... Massive splenomegaly Marrow fibrosis (dry tap) Filamentous projections on LM Stains TRAP+ CD20+ (B-cell) ```
HAIRY CELL LEUKEMIA Treat with... Cladribine Pentostatin
109
Anemia Thrombocytopenia Neutropenia Myeloblasts (basophilic cytoplasm, almost no nuclei) Auer rods - Myeloperoxidase+ cytoplasmic inclusions that are azurophilic and formed by the fusion of primary granules Pancytopenia DIC Associated with... Prior exposure to alkylating chemotherapy or radiation Myeloproliferative disorders Down syndrome Note - May present with DIC
ACUTE PROMYELOCYTIC LEUKEMIA (M3-AML) t(15;17) - Responds to all-trans retinoic acid inducing differentiation of promyelocytes PML/RARalpha gene produced retinoic acid receptor alpha gene on chromosome 17
110
``` Adult or elderly with... Splenomegaly Anemia Leukocytosis with left shift (leukocyte differentiation with a left shift) Thrombocytosis ``` Maturing granulocytes (vs blasts in AML) Low leukocyte alkaline phosphatase (LAP) May progress to AML or ALL (blast crisis) Note - High LAP in leukemoid reaction
CHRONIC MYELOGENOUS LEUKEMIA (CML) Philadelphia chromosome t(9;22) BCR-ABL1 that encodes a constitutively active tyrosine kinase Responds to bcr-abl tyrosine kinase inhibitor (Imatinib) Note - JAK2 mutation negative unlike other chronic myeloproliferative disorders
111
Child with... Lytic bone lesions Skin rash Recurrent OM with mastoid bone mass Birbeck ("tennis racket") granules/rods on EM S-100+ (mesodermal origin)
LANGERHANS CELL HISTIOCYTOSIS
112
Intense itching after hot shower Erythromelalgia (burning pain, red-blue coloration) due to episodic blood clots in vessels of extremities Increased RBCs/Hct Decreased EPO Leukocytosis Thrombocytosis
POLYCYTHEMIA VERA Always JAK2+ Treat with ASA
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Bleeding Thrombosis Plentiful, large, abnormal platelets on peripheral smear Erythromelalgia
ESSENTIAL THROMBOCYTHEMIA Massive proliferation of megakaryocytes and platelets Note - May be JAK2+
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Massive splenomegaly Hepatomegaly Teardrop RBCs Dry tap
PRIMARY MYELOFIBROSIS Obliteration of bone marrow with fibrosis due to increased fibroblast activity Note - May be JAK2+
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Increased RBC mass Decreased O2 sat Increased EPO
APPROPRIATE ABSOLUTE POLYCYTHEMIA Due to lung disease, CHF, or high altitude Note - Malignancy and Hydronephrosis present with inappropriate absolute polycythemia (normal O2 sat, ectopic EPO)
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Mechanism of heparin induced thrombosis and thrombocytopenia
HIT Development of IgG antibodies against bound platelet factor 4 (PF4) - antibody-heparin-PF4 complex activates platelets
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Mutation affecting metabolism of Warfarin
Polymorphism in vitamin K epoxide reductase complex (VKORC1)
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Mechanism of Warfarin induced skin necrosis
Protein C/S affected early leading to transient early microthrombosis - prevent with Heparin bridge
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Failure of PT to correct with Vit K supplementation
LIVER FAILURE Factor VII is affected first Note - Factor VIII is only factor made by endothelial cells rather than the liver (abnormal aPTT, normal PT)
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O2 content, SaO2, PaO2 in... ``` CO poisoning CN poisoning Anemia (reduced Hgb) Polycythemia (increased Hgb) High altitude ``` Note - O2 content = Hgb*SaO2 + PaO2
``` Decreased, Decreased, Normal Normal, Normal, Normal Decreased, Normal, Normal Increased, Normal, Normal Decreased, Decreased, Decreased ```
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Mechanism of uremic bleeding - Increased BT
Qualitative platelet dysfunction due to uremic toxins - PT, aPTT, and platelet count should be normal
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Causes of Pure Red Cell Aplasia (PRCA) - Severe hypoplasia of marrow erythroids with normal granulopoiesis and thrombopoiesis
Thymoma ALL B19
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Electrolytes in tumor lysis syndrome
Hyperkalemia Hyperuricemia Hyperphosphatemia Hypocalcemia

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