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Hemato/Onco 1.1

UWORLD Hematology & Oncology Review

Cutaneous lymphatic drainage below the umbilicus, including the anus, is done by the ___________________________, except for the ________________ and ____________________, which drain to the _________ -___________ nodes. Superficial Inguinal Lymph nodes; Glans penis and Posterior calf; Deep inguinal nodes.
Sickle cell patients are functional ___________________. Asplenics
Asplenics patients have increased risk of? Infection by ENCAPSULATED organisms
Which are encapsulated organisms? Pseudomonas aeruginosa, Strep pneumoniae, H. influenza type B, Neisseria meningitidis, E. coli, Salmonella, Klebsiella pneumoniae, and Group B streptococcus
What is the most common cause of Osteomyelitis in Sickle cell patient? Salmonella infection
Sickle cell patient with salmonella infection is higher risk of developing? Osteomyelitis
Functions of vWF: 1. binds to glycoprotein 1b receptors 2. carriers Factor 8
vWF deficiency results in: BT prolongation and +/- PTT may be prolonged
Treatment with __________________ stimulate the release of __________ for ________________. Desmopressin; von Willebrand factor; epithelium
Hereditary bleeding disorder von Willebrand disease
What is the main triad of symptoms seen in HUS? 1. Acute kidney injury --> Jaundice and elevated Cr 2. Microangiopathic Hemolytic Anemia 3. Thrombocytopenia
What are the labs seen in HUS? Decreased levels of Hb and PC, and increased levels of BT, lactate dehydrogenase, bilirubin, and BUN and Cr.
Most common organisms that cause HUS? 1. Shiga-like toxin 2. E. coli O157;H7
Schistocytes are associated in which disorders? Microangiopathic hemolytic anemias, including DIC, TTP/HUS, HELLP syndrome, mechanical hemolysis (heart valve prosthesis)
Fragmented RBCs Schistocytes
X-linked recessive disorder in affected males produce: 1. UNAFFECTED SONS as they only receive Y chromosome 2. CARRIER DAUGHTERS as they only receive is X chromosome
What are the 9 most common XR disorders? 1. G6PD deficiency 2. Fabry disease 3. Wiskott-Aldrich Disease 4. Ocular albinism 5. Hunter disease 6. Bruton agammaglobulinemia 7. Hemophilia A and B 8. Lesch-Nyhan Syndrome 9. Duchene (Becker) muscular dystrophy
Steps in development of inflammation 1. Margination, 2. Rolling, 3. Activation, 4. Tight Adhesion and Crawling 5. Transmigration
Which step in inflammation involves neutrophils rolling into endothelium via losing PSGL-1, to L-selecting or to E-selectin/P-selectin. Rolling
PECAM-1 protein is involved in _______________________. Transmigration
Neutrophils migrate out of vasculature by squeezing between cells via the integrin and adherence to PECAM-1. Transmigration
LAD Type 1 disease is a deficient in which step of inflammation? Transmigration
Recurrent skin infection without PUS formation, delayed detachment of umbilical cord, and poor wound healing. Dx? LAD type 1 disease
ICAM-1 protein is involved in ________________________, process of inflammation. Tight adhesion and crawling
Mar-1 and LFA-1 are ______________________________, which have neutrophils then attached to the __________________ on endothelial cells. CD18 B-2 integrins; ICAM-1
Passage of circulating inflammatory leukocytes into damaged tissue. Inflammation
What are the acute phase reactants involved in inflammation? Ferritin, Fibrinogen, Serum Amyloid A, Hepcidin, C-reactive protein.
What process is responsible for eliminating T-cells that bind to self or MHC antigens with overly high affinity? Negative Selection of T-cells
Where does negative selection occur? Thymic medulla
Failure of ________________ selection often is the process of developing autoimmune disorders. Negative
Which cells are created in negative selection? Cytotoxic and Helper T cells
Which cells are to survive Positive Selection? T-cells that express TCR.
A lack of TCR on a cell means ________________ Apoptosis
Positive selection occurs ___________________ negative selection and it is located in the _________________________. Before; Thymic Cortex
Which process is designed to create cells that recognize self? Positive Selection
Benign leukocytosis (>50,000) that occurs in response to underlying condition such as severe infection Leukemoid Reaction
Dohle bodies and Increased bands (N=3-5%), early mature neutrophil precursors (myelocytes) Seen in PBS of Leukemoid reaction
Elevated LAP is seen in ________________________. Leukemoid Reaction
Allopurinol inhibits ____________________, leading to an increased number of active metabolites. Xanthine oxidase (XO)
________________ is converted into active metabolites via the ______________ pathway and aided by _____________________ (enzyme) 6-Mercaptopurine; TPMT pathway; Xanthine oxidase
Common histochemical marker for Epithelial cell carcinomas Cytokeratin
What is keratin made of? Glycine, which allows for the formation of H-bonds, and Cysteine with form sulfide bonds, increasing cell stability and strength.
p53: tumor suppressor gene that controls cell division and apoptosis
In which stage of the cell cycle division does p53 act? G1 phase
p53 mutation causes? cell to advance to mitosis and keeps cell arrested in G1 phase, thus preventing apoptosis.
What syndrome or condition is associated with p53 mutation? Li-Fraumeni syndrome
Li-Fraumeni is a collection of __________________, which include _____________, _____________, ______________, and ________________. Malignancies; Sarcoma, Breast, Leukemia, and Adrenal gland
Another way to refer to Li- Fraumeni syndrome is? SBLA syndrome
p53 activates _______, blocks ______ phase ---> ______ phase activates p21; G1-phase --> S-phase
bcr-alb: FUSION gene; associated with CML
Associated gene in CML bcr-alb
Ras is a ___________________. Associated with the __________________ associated pathway. proto-oncogene; MAP - Kinase pathway
What does Erb-2 encodes for? Protein with Tyrosine kinase activity
Erb-2 is associated with _________________ and ________________ cancers. Breast and Ovarian
N-myc is an ________________________, associated with _______________. Proto Oncogene; Neuroblastoma
Burkitt's Lymphoma C-myc
C-myc is the gene associated with ____________________________, while N-myc is associated with __________________________. Burkitt's Lymphoma; Neuroblastoma
Repeated splenic infarction lead to a functional asplenic patient, which is seen in? Sickle Cell disease
Replacement of glutamic acid on position 6 of hemoglobin B-chain, by Valine. Dx? Sickle cell anemia
Sickle cell anemia is seen producing with type of anemia? Intrinsic Hemolytic anemia with Extravascular and Intravascular hemolysis.
TWO Hemoglobin S genes leads to ---> Sickle Cell anemia
How many Hb S gens are seen in Sickle cell disease? ONE Hb S gene
Sickle cell patients have increased risk of developing an Aplastic crisis by the infection of which microbe? Parvo B19
Salmonella osteomyelitis is most often seen in _______________ patients. SIckle cell
What is the treatment of Sickle cell anemia? Hydroxyurea (to increase HbF) and proper hydration
Sickle cells are Crescent-shaped RBCs
What factors precipitate Sickle cell anemia? 1. Low O2 2. High altitude 3. Acidosis (decreased pH)
__________________ mode of action, causes inhibition of Proteins ____ and _____, leading to _____________________, especially if the patient has deficiency of those two proteins. Warfarin; Proteins C and S; Skin necrosis
What are the 2 main MoA seen with Warfarin? 1. Inhibition of vitamin K dependent gamma-carboxylation of clotting factors 2, 7, 9, 10. 2. Decrease carboxylation of Proteins C and S
Which Vitamin K dependent protein has a very short half life? Protein C; it will be depleted in 1 day
How is warfarin-induced skin necrosis prevented? Warfarin is started with Heparin, construct a "Heparin-bridge" to prevent skin necrosis caused by Warfarin.
Which is the long term anticoagulant, Heparin or Warfarin? Warfarin
Warfarin acts on the _________________ coagulation pathway, and follows _______. Extrinsic ; PT
Warfarin site of action is at the ________, and is considered a ______________. Liver; Teratogen
What is used to reverse Warfarin toxicity? Vitamin K (Slowly); FFP (rapid reversal)
What is monitored while using Heparin? PTT and the intrinsic pathway
Purpose of the Heparin-bridge while starting patient on Warfarin Reduce the risk of recurrent venous thromboembolism and skin/tissue necrosis
How is CN poisoning clinically presented? Reddish discoloration, tachycardia, nausea, vomiting, confusion and weakness.
What is the main treatment of CN poisoning? NITRATES, as they are OXIDIZING agents
CN poisoning treatment induces ____, which has Fe____ instead to Fe____, leading to CN to attach to _______. Methemoglobinemia; Fe3+ instead of Fe2+; CN attaches to Fe3+ Leads to an overall decrease [CN]
CN toxicity treatment in steps: 1. Induce Methemoglobinemia by giving nitrates that will trap the CN. 2. Following, give Thiosulfates to convert CN to thiocyanate and be renally excreted
Patient presents with dark blue -purple lips and bluish skin discoloration, and with chocolate-colored blood. Dx? Methemoglobinemia
Treatment for methemoglobinemia Methylene blue and vitamin C
Which water soluble vitamin is often used to treat methemoglobinemia? Vitamin C
Which compound binds 200x times stronger to Hb than oxygen? Carbon monoxide (CO)
CO bound to HB instead of O2 is? Carboxyhemoglobin
Carboxyhemoglobin causes: 1. Decreased oxygen-binding capacity with Left shift in O2-Hb dissociation curve 2. Decreased O2 unloading into tissues
What type of anemia is found in HUS? characteristic RBCs on PBS? Hemolytic anemia with Schistocytes
MCC of TTP? Decreased level of ADAMTS13 (metalloproteinase)
What is the role of ADAMTS13? Cleavage of vWF multipliers
Hemolytic anemia, Thrombocytopenia, Fever, Neurological deficits, Renal disease are? Pentad of symptoms seen in TTP
What are the most important or relevant symptoms of TTP? 1. Hemolytic anemia (Elevated LDH, decreased Hepcidin), Schistocytes 2. Thrombocytopenia (Increased BT, normal PT/PTT)
Treatment for TTP? 1. Plasma exchange 2. Glucocorticoids 3. RITUXIMAB
Large vWF multipliers --> Increased platelet adhesion --> Increased platelet aggregation and thrombosis. Description of the pathogenesis of? TTP (Thrombotic Thrombocytopenic Purpura)
Defective platelet plug formation. Dx? Bernard-Soulier syndrome
Glanzmann thrombasthenia Defect in platelet integrin (GpIIb/IIIa)
Decreased GpIb is seen in ? Leads to? Bernard-Soulier syndrome; leads to defective platelet -to-vWF adhesion
Platelet disorder seen with LARGE platelets Bernard-Soulier syndrome
What platelet disorder exhibits abnormal or defective platelet-to-platelet aggregation? Glanzmann thrombasthenia
Autoimmune platelet disorder? Immune thrombocytopenia
Anti-gpIIb/IIIa antibodies are seen in: Immune thrombocytopenia
Increased levels of MEGAKARYOCYTES in blood smear Immune thrombocytopenia
Splenic macrophages "eat" or destroy the platelet-antibody complex. Pathogenesis seen in_____________________. Immune thrombocytopenia
What is the composition of Anti-Rh immune globulin> IgG anti-D antibodies, that OPSONIZE Rh+ fetal RBCs.
What cells prevent the Rh sensitization seen in Hemolytic disease of the newborn? Reticuloendothelial macrophages clear the affected Rh+ fetal RBCs, and prevents maternal Rh sensitization
The Rh (-) mother, during the first pregnancy produces _______ antibodies, which cannot cross the placenta. IgM
On second pregnancy of Rh(-) mother with Rh+ fetus, the mother has created _________________ antibodies, which are ______, and may cross the placenta and cause ___________________. anti-RhD antibodies; IgG; Hemolytic Disease of the Newborn
Clinical presentation of Hemolytic Disease of the Newborn? Jaundice, Kernicterus, Hydrops fetalis
XR disorders, classically seen hemarthrosis, intramuscular hematomas, and intracranial hemorrhage. Labs present increase in activated PTT, and normal PT and PC. Hemophilia 8 and 9
Hemarthrosis? bleeding behind the knee. Associated sign of Hemophilia
Desmopressin is used in the treatment of which Hemophilia? Hemophilia 8
Coagulation disorders include; 1. Hemophilia 8, 9, and 11 2. Vitamin K deficiency
Which parameter is always elevated in Coagulation disorders? PTT
Why is PTT always elevated in Vitamin K deficiency and in all Hemophilias? Affection of the Intrinsic coagulation Pathway yields an increase in PTT
PT is prolonged by affecting the ________________________ coagulation pathway. Extrinsic
PTT is prolonged by affecting he _____________________ coagulation pathway. Intrinsic
Which factors form part of the Extrinsic coagulation pathway? Factor 7
Factors 12, 11, 9, 8 are part of the _______________ coagulation pathway. Intrinsic
DiGeorge syndrome exhibits? Extreme deficiency in mature T-lymphocytes, which manifest in poor development of Lymph Node Paracortex
Failure of development of the 3rd and 4th branchial pouches is seen in: Di George syndrome
What is agammaglobulinemia? Absence of B-cells, preventing primary lymphoid follicles and germinal centers from forming in the Lymph Node Cortex
Which part of the Lymph node is affected in Di George syndrome? Paracortex
Paracortex is undeveloped in ____________________ syndrome. Di George
The cortex of the lymph node is affected and thus produces? Agammaglobulinemia
Activation of the ______ leads to _____________________. Rb ; Hypophosphorylation
Mutated Rb results in? inactive or Hyperphosphorylated Rb
A hyperphosphorylated Rb gives rise to: 1. Retinoblastoma 2. Osteosarcoma
Which RNA polymerase is restricted to the nucleus? RNA pol 1
RNA pol 1 role? Essential for RIBOSOMAL compounds
RNA pol 1 encodes for? rRNA
RNA pol 2 encodes for which forms of RNA? mRNA, snRNA, and microRNA
What is the role of mRNA? Translation at ribosomes to form proteins
Involved in mRNA splicing and transcription regulation snRNA
Which type of RNA causes gene silencing? By what RNA pol is it encoded? RNA pol II encodes for microRNA which is responsible for gene silencing
RNA pol III encodes for which 2 types of RNA? tRNA and 5srRNA
Adaptor molecule linking codons with specific amino acids tRNA
Common complication of C. trachomatis or N. gonorrhea sepsis, or acute Pancreatitis, or burn injury is? Disseminated Intravascular Coagulopathy (DIC)
DIC labs show elevated levels of __________________________, and a decrease in __________. Increased: PT, PTT, BT, and D-dimer Decreased: PC, fibrinogen, and factors 5 and 8
Acanthocytes are seen in: Liver disease and in Abetalipoproteinemia
How else are acanthocytes referred as? "Spur" cell as they are pointy or spike cells
Proper name for "Bite cells" Degmacytes
Bite cells are seen in ________________________. G6PD deficiency
Which cells are seen in HbC disease, Asplenia, Liver disease, and Thalassemias? Target cells
HALT Mnemonic used to describe disorders/conditions that present Target cells
Small, spherical cells without central pallor Spherocytes
Spherocytes are associated to which pathologies? 1. Hereditary spherocytosis 2. Drug-and-infection induced hemolytic anemia
Another name for Echinocytes? Burr cells
Echinocytes or Burr cells are seen in: End-stage renal disease and Pyruvate Kinase deficiency
What disorders present Basophilic Stippling? Sideroblastic anemias (lead poisoning, myelodysplastic syndromes), thalassemias
The aggregation of residual ribosomes is known as ________________________. Basophilic stippling
BCL-2 is associated with _____________________ lymphoma. Follicular
What is the translocation associated with Follicular lymphoma? Gene involved in Follicular lymphoma? t(14:18); bcl-2 gene
Development of Hyperphosphatemia, Hypocalcemia, hyperkalemia and Hyperuricemia, in the treatment of lymphoma, are due to? Tumor Lysis syndrome
What drugs are used in chemotherapy of a Leukemia or Lymphoma, in order to prevent Tumor Lysis syndrome? Allopurinol and Rasburicase
Allopurinol is an _______________ inhibitor,and Rasburicase is a ___________________ inhibitor. Xanthine oxidase (XO) ----- Allopurinol Urate oxidase (UO) ---------- Rasburicase
What enzymes are used during Purine Catabolism pathway? First Xanthine oxidase is used to form Uric acid form Hypoxanthine. Then, urate oxidase, converts uric acid into allantoin.
Common LMWH Enoxaparin
Enoxaparin activated ______________ which then binds to ___________, which causes a halt in the conversion of _______________ to ________. Antithrombin III; Factor Xa; Prothrombin to Thrombin
What is the common symptom triad seen in Aplastic anemia? 1. Low Hb 2. Absent hematopoietic cells in Bone marrow 3. Thrombocytopenia
The low levels of Haptoglobin seen in aplastic anemia, lead to the development of _______________________________. Intravascular hemolysis
Low haptoglobin ---> Intravascular hemolysis
Increased LDH ---> Hemolytic anemia
Low Hb and Increased EPO synthesis ---> Aplastic anemia
If aplastic anemia presents with Increased levels of EPO and LDH, and low levels of Hb and haptoglobin, thus it describes the pathology as? Aplastic HEMOLYTIC anemia, with INTRAVASCULAR hemolysis
Decreased Haptoglobin is seen in: 1. Intravascular hemolysis (Hereditary spherocytosis, PK deficiency, autoimmune hemolytic anemia, transfusion reactions) 2. Extravascular hemolysis (intraperitoneal hemorrhage) 3. Intramedullary hemolysis (thalassemias, sideroblastic anemia, megaloblastic anemia) 4. Burns, pregnancy, and genetics
Elevated Haptoglobin (>40) is associated with which pathologies? 1. Diseases with an elevated ESR (trauma, infection, hepatitis, amyloidosis, collagen diseases, lymphoma, and leukemia) 2. Obstructive biliary diseases 3. Dry, aplastic anemia 4. Increased estrogen 5. Nephrotic syndrome
Haptoglobin binds to_____________________________, causing inhibition of oxidative activity. Free hemoglobin
What the most two common side effects of OPIOID therapy? Constipation and Miosis
What receptors are used by opiods? Mu receptors
Opioid therapy causes stimulation of the ______ receptors in the ______ tract, causing a __________________ in secretions and __________________. Mu receptors; Gastrointestinal tract; decrease; Gastric mobility
Leukemoid reaction has a similar presentation to? CML
WHat is the clinical presentation seen in Leukemoid reaction and CML? Leukocytosis and an imbalance of LAP.
CML has an ____________________ LAP, while in Leukemoid reaction, the LAP is ___________________. CML---- decreased Leukemoid reaction ---- normal
CML diagnosis is done by: 1. Philadelphia chromosome t(9;22) 2. BCR-ALB fusion gene 3. mRNA 4. Decreased level of LAP 5. Leukocytosis
t(9;22) Philadelphia Chromosome (CML)
In which highly proliferative malignancy is the Philadelphia chromosome associated? CML
Inflammatory changes in lymph node, which can be either reactive hyperplasia and malignant transformation Lymphadenopathy
Reactive Hyperplasia is classified by: 1. Follicular hyperplasia (follicles increase in size and number) 2. Sinus Hyperplasia (sinuses enlarged and fill with Histiocytes) 3, Diffuse hyperplasia (normal structure, diffused with sheets of lymphocytes immunoblasts and macrophages
Polygonal response of lymph node cells is seen in? Reactive Lymphoid Hyperplasia
Proliferation of different cell types within the lymph node Reactive Lymphoid Hyperplasia
Different stimuli --> different cell-lines Reactive Lymphoid Hyperplasia
Monoclonal TCR Malignant transformation
Uncheck proliferation of 1 cell line Malignant transformation
"Starry-sky" appearance Burkitt's Lymphoma
t(8;14) Burkitt's Lymphoma
c-MYC Burkitt's Lymphoma
Jaw lesion (mass) most commonly seen in young adults. Endemic in Africa Burkitt's Lymphoma
Burkitt Lymphoma is associated with ________________ (virus) EBV
Tingible body macrophage is often seen in histological view of: Burkitt's Lymphoma
t(11:14) is associated with: Mantle Cell lymphoma
Mantle cell lymphoma shows translocation __________________, which is a mutation of the __________________________. t(11;14); cyclin D1 locus
What aspect to the cell cycle is affected in Mantle cell lymphoma? G1 to S-phase transition
Most common type of NON-Hodgkin lymphoma in adults Diffuse large B-cell lymphoma (DLBL)
What genetic alterations are seen in Diffuse large B-cell lymphoma? Bcl-2 and Bcl-6
Very aggressive lymphoma, CD5+, and usually presents in late-stage disease. Mantle cell lymphoma
Indolent course (Non-Hodgkin) Follicular Lymphoma
Painless "waxing and waning" lymphadenopathy Follicular Lymphoma
Which Non-Hodgkin Lymphoma is associated with states of chronic inflammation? Marginal zone Lymphoma
Marginal zone Lymphoma is associated to which common disorders? Sjogren syndrome and chronic gastritis (MALT lymphoma)
NH B-cell lymphoma seen most commonly in adult MALES? Mantle Cell lymphoma
Primary CNS lymphoma is considered an __________________. AIDS-defining illness.
Solitary or single ring enhancing lesion in on the MRI of an HIV + patient, indicates? Primary CNS lymphoma
Non-Hodgkin T cell lymphomas are: 1. Adult T-cell lymphoma 2. Mycosis fungoides/ Sezary Syndrome
What neoplasm is associated with HTLV-1 and IV drug abuse? Adult T-cell lymphoma
Japanese male with Hx of IV drugs use, presents cutaneous lesions, and a lab panel showing hypercalcemia. X-ray of bone show Lytic bone lesions Adult T-cell lymphoma
Skin patches/plaques; CD4+ cell with "cerebriform" nuclei and intraepidermal neoplastic cell aggregates Mycosis fungoides/ Sezary Syndrome
Mycosis fungoides may progress to ___________________. Sezary Syndrome (T-cell leukemia)
Clinical signs of Megaloblastosis? Acute/chronic pancreatitis, hepatomegaly, +/- jaundice, Megaloblastic anemia due to Vitamin B12 deficiency
Vitamin B12 deficiency: Common cause Megaloblastic anemia, as it impairs the synthesis of Purine and Pyrimidine bases
What is the difference in symptomatology between megaloblastic anemia caused by Vit B12 deficiency and Folate deficiency? Vitamin B12 deficiency cause neurological symptoms, wand Folate deficiency does not produces any neurological symptoms.
What are the reasons/causes of the rapid increase in cell death during radiation therapy? 1. Increase breakage of dsDNA structures 2. Increase oxygen free radicals
What is a key characteristic of Sickle cell TRAIT? Protection against Malaria caused by P. falciparum
Features of Sickle cell Trait: 1. NO symptoms of Sickle cell anemia 2. No change in life expectancy 3. Inherited protection against P. falciparum malaria 4. HbA > HbS
HFE protein mutation: Hemochromatosis
What are the resulting effects of Hemochromatosis' mutated HFE? 1. Decrease in Hepcidin synthesis by hepatocytes and, 2. Increase DMT1 expression by enterocytes, 3. IRON OVERLOAD
Which condition is defined or characterized by Iron overload? Hemochromatosis
What are some complications seen in Hemochromatosis? Heart failure, "Bronze" diabetes, and increased risk of developing HCC.
What is the PRIMARY iron lab disturbance in Hemochromatosis? Elevated serum iron levels
Hemochromatosis is associated with which two kind of cardiomyopathies? Dilated and Restrictive
Key regulator of the ENTRY of iron into circulation Hepcidin
High levels of Hepcidin would lead to _______________________, as more _____________ gets trapped inside cell and not transported out. Anemia; Iron
Low levels of Hepcidin mean? There is nothing impeding the efflux of iron from enterocytes into circulation, thus increasing gut iron absorption, ultimately leading to iron overload, as seen in hemochromatosis.
Angiogenesis is induced by: VEGF and FGF
VEGF and FGF are key stimulation of ___________________ seen in neoplastic and granulation tissue. Angiogenesis
VEGF function: Acts on many tissues, and increases motility and proliferation, leading to new capillaries
involved in endothelial cell proliferation, migration, and differentiation. FGF
What cytokines promote the expression of VEGF? IL-1 and INF-g
Missense mutation in position 6 of Hb B-chain. Sickle cell disease
GAG ---> GTG Sickle cell disease
Sickle cell disease Glutamic acid replaced by Valine in position 6 of Hb B-chain
What are the skin, GI, pulmonary and cardiovascular manifestations of Carcinoid syndrome? Skin flushing, GI watery diarrhea, pulmonary bronchospasm, and cardiovascular valvular, fibrous plaque formation
Octreotide? MC treatment of Carcinoid syndrome
Elevated 5-HIAA in 24 hour urine collection often indicates? Carcinoid syndrome
Octreotide is an _________________________ analogue, used to treat _________________ syndrome. Somatostatin analogue; Carcinoid
Deficiency of Porphobilinogen deaminase leads to Acute Intermittent Porphyria (AIP)
AIP is due to deficiency in ______________________ Porphobilinogen deaminase
Abdominal pain, peripheral neuropathy, neuropsychological deficits, and reddish (port-wine)-brown urine Symptoms of AIP
WHat is the treatment of AIP? IV glucose to downregulate ALA synthase
AIP treatment with glucose downregulates what enzyme? ALA synthase
What is the old name for Porphobilinogen deaminase? Uroporphyrinogen I synthase
What is products are accumulated in AIP? Porphobilinogen and ALA
5 P's of AIP: 1. Painful abdomen 2. Port-wine -colored urine 3. Polyneuropathy 4. Psychological disturbances 5. Precipitated by drugs (CYP450 inducers), starvation, alcohol
Ferrochelatase and/or ALA dehydratase are inhibited in: Lead Poisoning
Increased serum ALA levels and accumulated Protoporphyrin, indicated what diagnosis? Lead Poisoning
Lead poisoning leads to development of _______________ anemia, GI and kidney disease. Microcytic
Basophilic stippling in PBS and ringed sideroblasts in bone marrow. Macrocytic anemia in lead poisoning
Blistering cutaneous photosensitivity is seen in? Porphyria cutanea tarda
Which porphyria is associated to Hepatitis C virus infection? Porphyria cutanea tarda
What is the MC porphyria? Porphyria cutanea tarda
Enzyme mutated/deficient in PCT? Uroporphyrinogen decarboxylase
PCT and AIP are both autosomal __________________________ disorders. Dominant
Which heme synthesis enzymes act/work in the MITOCHONDRIA? Ferrochelatase and ALA synthase
Which enzyme is the rate-limiting step in heme synthesis? ALA synthase
Which ALA enzyme is deficient/mutated in Lead poisoning? ALA dehydratase
ALA dehydratase, Porphobilinogen deaminase, and Uroporphyrinogen decarboxylase, all work in the? Cytoplasm
The replacement of glutamic acid by Valine in Sickle cell disease, promotes the? Hydrophobic intearaction among Hb molescus leading oth HbS polymerization and erythrocyte sickling
What factors are promoters of RBC sickling? 1. Low O2 levels 2. Increased acidity (decreased pH) 3. Dehydration
Another term used for FIBRINOLYTICS? Thrombolytics
Alteplase, reteplase, streptokinase, are all examples of? Thrombolytics
What can inhibit Fibrinolytics directly? aminocaproic acid
Bone marrow failure caused by Hematopoietic stem cell deficiency. Describes the pathogenesis of ? Aplastic anemia
Fanconi anemia is known to be causative reason of developing _____________________ anemia Aplastic anemia
ParvoB19 and EBV are known microbial organisms that develop _____________ anemia. Aplastic anemia
What drugs are known to cause or promote development of aplastic anemia? Carbamazepine, Chloramphenicol, sulfonamides
What is a clinical sign of aplastic anemia? Pancytopenia
WHat is Pancytopenia? Decreased levels of Hb, platelet count, and low WBC levels.
In Pancytopenia, the laboratory abnormalities lead to what kind of clinical manifestations? Fatigue, weakness, and pallor by anemia, mucosal bleed, easy bruise, and petechiae by thrombocytopenia, and recurrent infection by the low WBC count.
Hypocellular bone marrow biopsy, composed of fat and stromal cells? Aplastic anemia
What is a key component of MDR1, that is related to chemotherapy tolerance? P-glycoprotein
What gene may be mutated during chemotherapy, leading to resistance of the treatment? MDR1
In hemochromatosis, there is accumulation of ________________, which is of _________________ or ______________ pigment. Hemosiderin; Yellowish or Brown pigment
______________ is very common among _____________ population and those ___________- +. G6PD deficiency; Black; HIV
What some factors that precipitate G6PD deficiency? 1. Infections 2. Drugs (Dapsone, Antimalarials, sulfonamides, 3. DKA, and 4. Favism
Important RBC findings on G6PD deficiency PBS? 1. BItce cells and, 2. Heinz bodies
Decreased in reduced glutathione level G6PD deficiency
African american patient presents with back pain, and hemoglobinuria few days after consuming fava beans. Dx? G6PD deficiency
What is the MC RBC enzymatic disorder? G6PD deficiency
G6PD deficiency is categorized as an ____________________ hemolytic anemia, exhibint _________________ and ______________ hemolysis. Intrinsic; Extravascular and intravascular hemolysis
Which are the intrinsic hemolytic anemias? Hereditary Spherocytosis, G6PD deficiency, PK deficiency, Paroxysmal nocturnal hemoglobinuria, Sickle cell anemia, and HbC disease.
Autoimmune hemolytic anemia is a ___________________ anemia. Extrinsic Hemolytic anemia
What is the clinical presentation of Hereditary Spherocytosis? 1. Hemolytic anemia, 2. Jaundice, 3. Splenomegaly
What are some common complications of Spherocytosis? Pigmented Gallstones, and Aplastic crisis due to B19 infection.
Increased MCHC, spherocytes, NEGATIVE Coombs test, and increased fragility on acidified glycerol lysis test of RBC. Dx? Hereditary Spherocytosis
Which condition is described by patient that bleeds, the coagulation cascade is activated, PT and PTT aer prolonged, as well as BT, and there is a decrease in fibrinogen, and an increased of D-dimer. DIC
TTP/HUS: Patients don't tend to bleed, only platelets are activated, tehr are normal levels of PT, PTT, and fibrinogen.
Hypercellularity of bone marrow is seen with: Myelodysplastic syndromes
AML subtype M3 Affects adults and is associated with DIC
t(15;17) AML subtype M3
PML/RARx fusion gene is associated with AML subtype M3
What is the treatment for PML? Trans-retinoid acid
Bone marrow failure and infiltration of RETICULOENDOTHELIAL system causing massive SPLENOMEGALY. Hairy cell leukemia
Hairy cell leukemia characteristics include: 1. DRY TAP -- unsuccessful bone marrow aspiration 2. Presence of lymphocytes with cytoplasmic (hair-like) progenctions
Unsuccessful bone marrow aspiration is known as, ______________, and it is associated with ___________________________. DRY TAP; Hairy cell leukemia
HER-2 is a ___________________ receptor. Extrepessin in 20% of _______ cancer. Its most common treatment is ___________________, a monoclonal antibody that down regulates cellular proliferation and promotes apoptosis. Tyrosine Kinase receptor; Breast Cancer; Trastuzumab;
What is the most common use for Trastuzumab? HER-2 (+) breast cancer and gastric cancer
Another name for Trastuzumab? Herceptin
Monoclonal antibody against HER-2 (c-erbB2) Trastuzumab
Which breast cancer drugs is also useful to prevent Osteoporosis in postmenopausal women? Raloxifene
What cancers are associated with Rb (tumor suppressor gene) mutation? Retinoblastoma and Osteosarcoma
EPO-stimulated hyperplastic marrow cell invasion of extramedullary organs, describes what process? Extramedullary Hematopoiesis
What is the MCC or MC condition in which extramedullary hematopoiesis is seen? Chronic hemolytic anemias such as B-Thalassemia
What is the physical characteristics seen in a patient with extramedullary hematopoiesis? Pathologic fractures in children, maxillary overgrowth and frontal bossing "Chipmunk" face
What conditions produce a "Chipmunk facie"? B-Thalassemia major, Bulimia nervosa, and Parotid swelling
Left renal thrombosis commonly develops? Varicocele
Nephrotic syndrome is considered a _____________________ state, leading to increase risk of renal thrombotic events. Hypercoagulable
What is used to stop the bleeding in a Hemophiliac patient? Thrombin
Hb precipitation seen in G6GP deficiency patients Heinz bodies
Basophilic nuclear remnants found in RBCs Howell-Jolly bodies
What conditions exhibit Howell-Jolly bodies? Hyposplenia and Aspenics
The reticulocyte count separates normocytic anemias into? Non-hemolytic and Hemolytic anemias
An elevated Reticulocyte count is defined as ________________%, and indicates __________________ anemias. > 2%; Hemolytic anemia
Which are the NON-hemolytic anemias? 1. Iron deficiency (early) 2. Anemia of chronic disease 3. Aplastic anemia 4. Chronic kidney disease
Hemolytic anemias are further divided into _______________ and ___________________. Intrinsic and Extrinsic
Intrinsic Hemolytic anemias are due to: 1. Membrane defects 2. Enzyme deficiencies 3. Hemoglobinopathies
1. Hereditary Spherocytosis 2. Paroxysmal Nocturnal Hemoglobinuria Intrinsic Hemolytic anemias caused by membrane defects
What are example of causes of Enzyme deficiencies resulting Intrinsic hemolytic anemias? 1. G6PD deficiency 2. Pyruvate kinase deficiency
Sickle cell anemia and HbC diseases are causative of _____________ hemolytic anemias, and classified as hemoglobinopathies. Intrinsic
Fanconi anemia, is a condition affecting the DNA repair, and classified as a ___________________ anemia. Megaloblastic anemia
Non-Megaloblastic anemias: 1. Diamond-Blackfan anemia 2. Liver disease 3. Alcoholism
What conditions/disorders cause Megaloblastic anemia due to defective DNA synthesis? 1. Vitamin B12 deficiency 2. Vitamin B9 (Folate) deficiency 3. Orotic aciduria
Defective Heme synthesis, yields what kind of anemias? Microcytic anemia
What are causative disorders that result in Microcytic anemia due to poor or defective heme synthesis? Iron deficiency (late), lead poisoning, sideroblastic anemia, and anemia of chronic disease
Defective globin chain, is seen with? Thalassemias, which result in Microcytic anemias
Hb Barts No alpha-globin; Hydrops fetalis
Created by: rakomi