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57 Cards in this Set

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Elderly Patient with Anemia and negative Fecal Occult Blood
Probably B12 deficiency 2° aging → gastric atrophy → [relative] achlorhydria → poor B12 release from eaten foods
#biochemistry #nutrition #gastrointestinal #hematology
Haptoglobin Levels
Haptoglobin Binds Free Hemoglobin within Blood Vessels

Complexed Hemoglobin-Haptoglobin is phagocytized by Spenic M∅

↓ serum haptoglobin levels ≈ intravascular hemolysis

normal serum haptoglobin levels rules out intravascular hemolysis (ie extravascular = direct splenic phagocytosis)
#hematology #diagnostics #molecules
Differentiating Intravascular from Extravascular Hemolysis
Haptoglobin Levels

Haptoglobin Binds Free Hemoglobin within Blood Vessels

Complexed Hemoglobin-Haptoglobin is phagocytized by Spenic M∅

↓ serum haptoglobin levels ≈ intravascular hemolysis

normal serum haptoglobin levels rules out intravascular hemolysis (ie extravascular = direct splenic phagocytosis)

NB: Extravascular includes Splenic Phagocytosis
#hematology #diagnostics #molecules
Diathesis Panel
Laboratory Abnormality of HUS
↑ Bleeding Time from Platelet consuming thrombi

oddly no ↑ PT, or PTT

Shiga toxin producing Shigella or E Coli O157H7
#microbiology #pathology #hematology
Warfarin Necrosis
Proteins C & S are natural anticoagulants

Protien C is vitamin K dependent and has much shorter λ than factors 2, 7, 9 and X.

Rare complication: in pts w/ already defective Protein C, warfarin creates a transient hypercoagulable state

Tx: Vitamin K + FFP
#hematology #pharmacology #toxicity
Hemosiderin
aggregation of ferritin micelles

maker of iron accumulation

golden yellow brown pigment

indicative of chronic iron overload/hemolytic anemia
#pathology #molecules #diagnostics #hematology
Pigment found in chronic iron overload
Hemosiderin

aggregation of ferritin micelles

golden yellow brown pigment

indicative of chronic iron overload/hemolytic anemia
#pathology #molecules #diagnostics #hematology
electrolyte abnormality following massive packed RBC transfusion
Hypocalcemia

Packed RBC's contain citrate anticoagulant

citrate chelates calcium
#pathology #electrolytes #hematology
Heparin vs LMWH
enoxapren = LMWH

both activate AT3 to bind Factors 9 X & 2

Only require pentasaccharide to bind AT3 & ↑ affinity

Heparin has >18 sacchardies, allowing it to wrap around AT3 and form a stable bond with factor 2 (ie thrombin)

LMWH cannot and thus has higher affinity for X than 2
#pharmacology #hematology
"Hotdog" looking inclusions in eosinophils
Major Basic Protein

Kills helminths
damages epithlium
#immunology
#hematology
Timeline for correcting B12 deficiency
Immediate ↑ Reticulocyte Count, drops off again

slow ↑ hemoglobin
#nutrition #hematology
Loss of FAS → SLE

pathogenesis?
impaired "Clonal deletion" of activated T's by CTL's in peripheral ts
#pathology #hematology #immunology
DOC: AML
t(15:17) RAR-PML

DOC: all-trans-retinoic acid
induces remission in 90% of pts
#hematology #pharmacology #chemotheraputic #neoplasia #pathology
many immature myelogenous cells on blood smear
Not AML: would be "blast cells" rather than immature cells (bands, metamyelocytes, myelocytes)

either CML t(9:22) or leukemoid rxn (ie infx)

depends on AP

low AP: CML
normal or high: leukemoid rxn
#pathology #diagnostics #hematology #neoplasia
Desmopressin
aka DDAVP

ADH analogue

used for central diabetes insipidus

and also coagulopathies
→ induces release of vWF & Factor 8 from endothelial cells (where they are produced and stored)
#pharmacology #drugs #hematology #nephrology
DDAVP
aka Desmopressin

ADH analogue

used for central diabetes insipidus

and also coagulopathies
→ induces release of vWF & Factor 8 from endothelial cells (where they are produced and stored)
#pharmacology #drugs #hematology #nephrology
DIC vs TTP vs HUS
DIC:
Pts bleed
Activated Coagulation Cascade
↑ PT & ↑ PTT
↓ fibrinogen, ↑ FDP (D-dimer)

TTP-HUS
usually do not bleed
Only Platelets activated
normal PT, PTT, fibrinogen, ∅ FDP
#hematology #pathology
Px of ITP
Ideopathic thrombocytopenic purprua

Bleeding from platelet destrx

Chidlren: acute and self-limited

Adults: insidious and chronic
#pathology #hematology #rheumatology
Nails which bow in and bend out
Spoon nails aka Koilonychia
specific for iron deficiency anemia

___________________

Plummer Vincent Sro: Triad of

Iron Deficiency Anemia
Glossitis
Esophageal Webbing (Dysphagia)
#pathology #hematology
Plummer Vincent Sro
Triad of

Iron Deficiency Anemia (Spoon Nails aka Koilonychia)
Glossitis
Esophageal Webbing (Dysphagia)
#pathology #hematology #gastrointestinal
Anemia + Dysphagia
Plummer Vincent Sro

Triad of

Iron Deficiency Anemia (Spoon Nails aka Koilonychia)
Glossitis
Esophageal Webbing (Dysphagia)
#pathology #hematology #gastrointestinal
Spoon Nails + Dysphagia
Plummer Vincent Sro

Triad of

Iron Deficiency Anemia (Spoon Nails aka Koilonychia)
Glossitis
Esophageal Webbing (Dysphagia)
#pathology #hematology #gastrointestinal
Spoon Nails
aka Koilonychia

Specific for Iron Deficiency Anemia

___________________

Plummer Vincent Sro: Triad of

Iron Deficiency Anemia
Glossitis
Esophageal Webbing (Dysphagia)
#pathology #hematology
Koilonychia
aka spoon nails

Specific for Iron Deficiency Anemia

___________________

Plummer Vincent Sro: Triad of

Iron Deficiency Anemia
Glossitis
Esophageal Webbing (Dysphagia)
#pathology #hematology
B6 deficiency anemia
required for first step of heme synthesis

microcytic hypochromatic B6 responsive anemia

same morphology as X linked Sideroblastic Anemia, but B6 responsive
#pathology #nutrition #hematology
Pure Red Cell Aplasia

Pathogenesis
Parvovirus B19

or

Thymoma
#pathology #hematology #microbiology #noplasia
Vitamin E deficiency
anti-oxidant which preserves cell membranes

most affected:
neurons (large surface area)
RBC's (poor antioxidant fnx)

manifestation:
Friederick's ataxia-like neuropathy w/ ataxi a & loss of proprioception and vibration +
hemolytic anemia
#pathology #nutrition #neurology #hematology
Teardrop RBC's
indicative of myelofibrosis or metastatic cancer

RBC's which had had to squeeze through fibrous strands

(not sliced like schistocytes)
#hematology #pathology #diagnostics
Acanthrocytosis
indicative of abetalipoproteinemia
or liver dz

no fat absorption
(no vitamin E absorption)
#hematology #pathology #diagnostics
Spur cells
extreme form of acanthrocytosis
or liver dz

indicative of abetalipoproteinemia
#hematology #pathology #diagnostics
Helmet Cell
Schistocyte
ie mechanical trauma
#hematology #pathology #diagnostics
bisphosphoglycerate mutase
bisphosphoglycerate mutase
present only in RBC's & Placenta
alternative glycolytic pathway which produces no ATP

BPG mutase: 1,3 BPG → 2,3 BPG

Phosphatase: 2,3 BPG → 3 Phosphoglycerate

Bypasses one step by Phosphoglycerate kinase which would have produced ATP

2,3 BPG allosterically induces a right shift → O2 dumping

[I guess BPG mutase fnx is upregulated by hypoxia]
#biochemistry #hemoglobin #molecules #energymetzm
1,3 BPG → 2,3 BPG
bisphosphoglycerate mutase
present only in RBC's & Placenta
alternative glycolytic pathway which produces no ATP

BPG mutase: 1,3 BPG → 2,3 BPG

Phosphatase: 2,3 BPG → 3 Phosphoglycerate

Bypasses one step by Phosphoglycerate kinase which would have produced ATP

2,3 BPG allosterically induces a right shift → O2 dumping
#biochemistry #hemoglobin #molecules #energymetzm
2,3-bisphosphoglycerate
bisphosphoglycerate mutase
present only in RBC's & Placenta
alternative glycolytic pathway which produces no ATP

BPG mutase: 1,3 BPG → 2,3 BPG

Phosphatase: 2,3 BPG → 3 Phosphoglycerate

Bypasses one step by Phosphoglycerate kinase which would have produced ATP

2,3 BPG allosterically induces a right shift → O2 dumping
#biochemistry #hemoglobin #molecules #energymetzm
CD55
Paroxysmal Nocturnal Hemoglobinuria
Neither Paroxysmal nor Nocturnal
Constant w/ ↑ visualization in first urine of day

mutation in PIG-A gene which codes for glycosylphosphatidylcholine (GPI) anchor necessary for CD55 and CD59 attachment

CD55 & 59 important to inactivate complement

a disorder of the stem cells → pancytopenia

chronic hemolysis → recurrent venous thromboses

>>deficiency of CD55 and CD59 diagnostic of PNH<<
#pathology #hematology
Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal Nocturnal Hemoglobinuria
Neither Paroxysmal nor Nocturnal
Constant w/ ↑ visualization in first urine of day

mutation in PIG-A gene which codes for glycosylphosphatidylcholine (GPI) anchor necessary for CD55 and CD59 attachment

CD55 & 59 important to inactivate complement

a disorder of the stem cells → pancytopenia

chronic hemolysis → recurrent venous thromboses

>>deficiency of CD55 and CD59 diagnostic of PNH<<
#pathology #hematology
CD59
Paroxysmal Nocturnal Hemoglobinuria
Neither Paroxysmal nor Nocturnal
Constant w/ ↑ visualization in first urine of day

mutation in PIG-A gene which codes for glycosylphosphatidylcholine (GPI) anchor necessary for CD55 and CD59 attachment

CD55 & 59 important to inactivate complement

a disorder of the stem cells → pancytopenia

chronic hemolysis → recurrent venous thromboses

>>deficiency of CD55 and CD59 diagnostic of PNH<<
#pathology #hematology
"Starry Sky" Lymphoma
Burkitt's Lymphoma

appearance from M∅ &apoptotic bodies

almost all assoc. w/ c-MYC translocations on chrom 8 usually w/ Ig on 14 t(18;14)
#pathology #neoplasia #hematology
Accute Intermittent Porphyria
Deficiency of HMB synthase (Uroporphyrinogen I Synthase)

responsible for condensing 2 PBG's → HMB aka uroporphyrinogen 1

1. Abodminal Pain, Neurologic Sx w/o Photosensitivity
2. Urine Darkens on standing
3. ALA & PBG-uria

precipitated by EtOH, Barbituates, Hypoglycemia, Phenytoin & Griseofulvin
relieved by: heme, glucose
#pathology #biochemistry #hematology
HMB synthase
responsible for condensing 2 PBG's → HMB aka uroporphyrinogen 1

Deficiency: Acute Intermittent Porphyria

1. Abodminal Pain, Neurologic Sx w/o Photosensitivity
2. Urine Darkens on standing
3. ALA & PBG-uria

precipitated by EtOH, Barbituates, Hypoglycemia, Phenytoin & Griseofulvin
relieved by: heme, glucose
#pathology #biochemistry #hematology
Sequence of Ig Isotype Switching
Requires interaction of B cell CD40 and T cell CD40L (CD154)

Once you go on to the next you cannot come back

M D G E A
#hematology #immunology
Mitotane
adrenocorticolytic used for adrenocortical carcinoma
#pharmacology #endocrine
Tryptase
Indicative of Mast Cell Degranulation
#physiology #hematology
Blood Product indicative of Mast Cell Degranulation
Tryptase
#physiology #hematology
Sickler with Macrocellular Anemia
Folic Acid Deficiency 2° to ↑ Bone Marrow Turnover
#pathology #hematology
HFE mutation
"High Fe" = hematochromatosis

protein on basolateral membrane of SI intestinal crypt cells

compelxes with β2 microglobulin and binds transferrin receptor to regulat endocytosis of transferrin/iron

intracellular [iron] ≈ systemic iron → suppresses iron uptke

mutation = hematochromatosis

Liver cirrhosis and hepatocellular carcinoma are the end stage
#pathology #hematology
Hematochromatosis
HFE "High Fe" Mutation

protein on basolateral membrane of SI intestinal crypt cells

compelxes with β2 microglobulin and binds transferrin receptor to regulat endocytosis of transferrin/iron

intracellular [iron] ≈ systemic iron → suppresses iron uptke

mutation = hematochromatosis

Liver cirrhosis and hepatocellular carcinoma are the end stage
#pathology #hematology
Systemic Mastocytosis
↑ Histamine Secretion leads to manifestations

↑ Gastric secretion inactivates pancreatic and intestinal enzymes → diarrhea & malabosprtion

↑ vasodilation → flushing, hypotension, tachycardia, syncope

urticaria/, pruritis, dematographism
#pathology #hematology
Ristocetin
activates GP Ib-IX receptors on platelets which bind vWF

good test to see if vWF deficient
#pathology #diagnostics #hematology
prolonged PTT c prolonged bleeding time, same pathology
vWF deficiency

vWF binds Factor8 and marketly ↑↑ λ
#pathology #hematology
pancytopenia s hepatosplenomegaly
aplastic anemia
--severe B12 or folic deficiency
--aleukemic leukemia
--myelodysplastic sro

"dry tap" hypocellular marrow filled with fat & fibrotic stroma
#pathology #hematology
Factor V Leiden mutation
glutamine for arginine substrition near protein C cleavage site → not degraded

hypercoagulative

[activated protein C (APC) responsible for inactivating factors V & 7]
#pathology #hematology
glutamine for arginine substrition near protein C cleavage site on Factor V
Factor V Leiden mutation → not degraded

hypercoagulative

[activated protein C (APC) responsible for inactivating factors V & 7]
#pathology #hematology
Reed Sternberg Cells
ample cytoplasm
double/bi-lobed nuclei

indicative of Hodgkin's Lymphoma
#pathology #neoplasia #hematology
Cell with double/bi-lobed nuclei and ample cytoplasm
Reed Sternburg Cells

pathognomic for Hodgkin's Lymphoma
#pathology #neoplasia #hematology
Hematologic/poietic Effects of Glucocorticoids
lyses lymphocytes, eosinophils and basophils, ↓ monocyte differentiation into M∅ (thus decreasing antigen presentation)

"↑" PMN's on CBC via demargination, does not actually ↑ # PMN
#pharmacology #physiology #hematology
Severe hypothermia and oxygen dyssociation curve
left shift (does not release O2 into ts as well)

this is because the lungs are normally cooler than the tissues, helpful in normal physiologic state to load with more o2
#biochemistry #hemoglobin