hema 1 Flashcards
asplenia and infection?
loss of splenic macrophage and Ab production–Increase the risk of encapsulated organism sepsis
what is the fun? of splenic macrophage and Ab production?
splenic macrophage(found in red pulp around cord and sinusoid--engulf blood microbes and aged RBC Ab production--From white bulb after Ag presentation?
acquired complement deficiency causes?
SLE and antiphospholipid syndrome
Androgen abuse drugs?
exogenios(Testostrone replacment)
systemic(stanozolol and nandronolol)
precursor(DHEA)
CM?
Reproductive/CVS/Psychiatric and haematologic
Reproductive?
Men-testicular atrophy. decrease sperm production and mild gynecomastia..
Female:acne, voice depning,hirtuism and AUB
CVS?
left ventricular hypertrophy
decrease HDL
Increase LDL
Psychiatric?
aggressive behavior
mood disturbance
haematologic
polycythemia
hypercoagulability
Differentials?
autologous B/D transfusion
erythropoietin injection
polycythemia vera
strenuous exersise
autologous B/D transfusion?
blood removed some week before and replace blood near competition.
have only polycythemia
erythropoietin injection?
only polycythemia
Multiple myeloma?
plasma cell disorder
common in elderly
CM?
symptom of anemia
Bone pain
punched out a lesion on an x-ray
Diagnosis?
> 10% plasma cell in BM biopsy(clock face nuclei cell with intracytoplasmic IgG)
Laboratory?
serum protein gap
RF
rouleaux forming RBC in pheripherial morphology
Hypercalcemia
normocytic anemia
urinalysis–Increase light chain(BJP) with the negative dipstick.
Anemia (normocytic, normochromic)
what is the serum protein gap?
total protin and albumin gap >=4g/dl
causes?
Multiple myeloma
Wanderstorm Macroglobulinemia
Connective tissue disease
Infection
How to D/T?
Plasma electrophoresis
How?
polyclonal/monoclonal
Polyclonal?
Connective tissue disease
Infection
Monoclonal(M spike)?
Multiple myeloma
Wanderstorm Macroglobulinemia
How to d/tMultiple myeloma from Wanderstorm Macroglobulinemia?
clinical
PEEP
Bone marrow biopsy
Complication
clinical?
WM has cx by -Peripheral neuropathy -No CRAB findings -Hyperviscosity syndrome: Headache Blurry vision Raynaud phenomenon Retinal hemorrhage
PEEP?
WM–IgM
MM-IgG.IgA
Bone marrow biopsy?
IN WM
>10% small lymphocytes with intranuclear pseudoinclusions containing IgM (lymphoplasmacytic lymphoma).
Complication?
MM-infection and amilodosis
WM-thrombosis
CRAB?
HyperCalcemia
Renal involvement
Anemia
Bone lytic lesions
The normal range os srum albumin?
3.4 to 5.4 g/dL (34 to 54 g/L).
The normal serum protein level?
6 to 8 g/dl.
Clinical future of paroxysmal nocturnal hemoglobinuria?
Fatigue
Dark urine(hemoglobinuria)
Jaundice
VTE(cerebral and abdominal)
Pathophysiology?
Loss of anchor proteins (GPI)–CD55 and DC59) absence – Cell enables to inhibit complement activation—Hemolysis
workup?
Coombs ⊝ hemolytic anemia(High LDH and Low haptoglobin) Pancytopenia Venous thrombosis CD55/59 negative RBCs on flow cytometry Hyperbilirubinemia
Treatment?
Eculizumab (targets terminal complement protein C5)
Iron and folate suplementation
CM of Budd-chiari syndrome?
acute/subacute/chronic
acute?
Jaundice Elevated transaminase Hepatic encephalophaty Varicial bleeding Elevated INR/PTT
subacute/chronic?
Progresive abdominal pain Hepatomegaly Spleenomegaly Ascitis Elevated bilirubin and transaminase
DIagnosis?
Abdominal dopler U/S
causes?
myloproliferative disease
malignancy(HCC)
OCP
PNH
Polycytemia vera?
Primary polycythemia. Disorder of RBCs, usually due to acquired JAK2 mutation.
CM?
aquaporic pruritis erythromelagia facial plutora transient vision disturbance thrombosis/heamorage HTN High RBC turnover(gouty arteritis)
aquaporic pruritis?
iching during shower
erythromelalgia?
Rare but classic symptom
(severe, burning pain and red-blue coloration)
due to episodic blood clots in vessels of the
extremities A .
Transient vision disturbance?
due to hyperviscosity
Treatment?
Phlebotomy
Hydroxyurea
Ruxolitinib (JAK1/2 inhibitor)
Blood transfusion related anaphlaxis?
Bronchospasm(RD)
Angioedema
Hypotension
Shock and respiratory failure
Managment?
epiniphrne
antihistamin and bronchodilator co administration
respiratory support/MV if RF developed
future transfusion–IgA deficient plasma and wahed RBC
How lymphoploliferative disease cause anemia?
Replacment of BM cell by cancerous cell
Hodgkin lymphoma?
Is a type of lymphoma, which is a cancer originating from white blood cells called lymphocyte
Epstein–Barr virus (EBV) may have an increased risk
Painless LDP
age affected?
early adulthood and >60
LN affected?
contagious
cervical
supraclaviculaar
mediasternal
CM?
Itchy skin Night sweats fever Unexplained weight loss Hepatosplenomegaly Pain following alcohol consumption(specific) Back pain Nephrotic syndrome(MCD) pruritis
managment?
Patients of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy.
Generaly have good prognosis
poor prognosis sighn?
Age ≥ 45 years
Stage IV disease
Hemoglobin < 10.5 g/dl
Lymphocyte count < 600/µl or < 8%
Male
Albumin < 4.0 g/dl
White blood count ≥ 15,000/µlDiagnosis?
biopsy–reed stunberg cell
PET scan with florodeoxy glucose
eosinophilia.elevated LDH
PET scan with florodeoxy glucose?
Uptake by high metabolic cell(tumour,brain,liver) and urinary tract due to excretion
The Reed–Sternberg cells are?
identified as large often bi-nucleated cells with prominent nucleoli and an unusual CD45-, CD30+, CD15+/- immunophenotype
abundant, amphophilic, finely granular/homogeneous cytoplasm
risk factor?
Sex: male
Ages: 15–40 and over 55
Family history
History of infectious mononucleosis or infection with Epstein–Barr virus, a causative agent of mononucleosis[
Weakened immune system, including infection with HIV or the presence of AIDS[23]
Prolonged use of human growth hormone
Exposure to exotoxins, such as Agent Orange
Risk factor for non hodgkin lymphoma?
Infection
Some chemicals, like polychlorinated biphenyls (PCBs),diphenylhydantoin, dioxin, and phenoxy herbicides.
Medical treatments, like radiation therapy and chemotherapy
Genetic diseases, like Klinefelter’s syndrome, Chédiak-Higashi syndrome, ataxia telangiectasia syndrome
Autoimmune diseases, like Sjögren’s syndrome, celiac sprue, rheumatoid arthritis, and systemic lupus erythematosu
Infection?
Epstein-Barr virus – associated with Burkitt’s lymphoma, Hodgkin’s lymphoma, follicular dendritic cell sarcoma, extranodal NK-T-cell lymphoma
Human T-cell leukemia virus – associated with adult T-cell lymphoma
Helicobacter pylori – associated with gastric lymphoma
HHV-8 – associated with primary effusion lymphoma, multicentric Castleman disease
Hepatitis C virus – associated with splenic marginal zone lymphoma, lymphoplasmacytic lymphoma and diffuse large B-cell lymphoma
HIV infection[6]
symptome?
Progresive,painless LDP
B-Symptome
Elevated LDH
How to d/t from TB?
TB LDP have rarely sytemic symptom unlike HLwhich have B symptome
Superior vena cava syndrome pathogenesis?
An obstruction of the SVC that impairs blood
drainage from the head (“facial plethora”;
note blanching after fingertip pressure in A ),
neck (jugular venous distention), and upper
extremities (edema). .
Commonly caused by?
Commonly caused by malignancy (eg, mediastinal mass, Pancoast tumor)
Thrombosis from indwelling catheters
fibrosing mediasternitis(TB and fungal infection)
whyit is Medical emergency?
Can raise intracranial pressure (if obstruction is severe)
headaches, dizziness,
risk of aneurysm/
rupture of intracranial arteries
adjuvant therapy?
Treatment given adition to standard therapy
consolidation therapy?
Given after induction therapy with multiple drug to reduce tumour burden
Induction therapy?
Intial treatment given rapideley to reduce tumour burden
maaintainance therapy?
given after initial and consolidation treatment to kill residual tumour cell
neoadjuvant therapy?
treatment given bevore standard treatment
salvage therapy?
Given after failure of primary regimen(in recurence)
To prevent long term disease progress and localize
risk of erethropoitin treatment?
moderate/sever HTN
nutrisional folate deficiency CM?
pancytopnia
macrocytic anemia
alcohol deficiency?
Increase excretion
Decreased absorbtion
Defct in storage
cause of anemia in SLE?
IDA
Animia of chronic disease
Heamolysis
other future?
lukopnia
thrombocytopnia
symptom of drug induced hemolytic anemia?
hemolysis
hyperbilirubinimia
reticulocytocis
splenomegaly
hemocystin uria and TE?
vascular damage
activation of cloting factor
inhibition of anticoagulation
Treatment?
pyridoxin
folic acid
documented deficoiency
TTP pathophysiology?
Decrease ADMAS level–uncleaved VWF–platelet activation.
Acquired or hereditary
clinical future?
Hemolytic anemia Thrombocytopenia Renal failure Neurologic symptom Fever
management?
Plasma exchange
Glucorticosteroid
Rituximab
mortality w/o plasma exchange?
90 %
HIV and NHL?
10 % risk
By leading EBV reactivation
Cause of macrocytosis?
megaloblastic
Non-megaloblastic
megaloblastic?
Folate deficiency B-12 deficiency orotic aciduria Drugs Fanconi
Drugs?
Hydroxyurea zidovudine phenytoin methotrexate sulfa drug
characteristics?
Hypersegmented neutrophil
low reticulocytosis
Anikocytosis and poikilocytosis
characteristics?
Hypersegmented neutrophil
low reticulocyte
Anikocytosis and poikilocytosis
Macroovalocyte(enlarged, oval-shaped erythrocytes)
Cxs?
non-hypersegmented neutrophil
low/normal/increased reticulocyte count
management?
First, do B12 and folate level then consider BM aspiration.
How to D/T lukomoid rxn from CML?
WBC count
Cause
LAP score
DOminant neutrophil precursor
WBC count?
LR=>50,000
CML=elevated (useualy > 100,00
Cause?
LR=Infection
CML=BCR-ABL mutation
LAP score?
LR=High
CML=Low
Dominant neutrophil precursor?
LR=more mature (metamyelocytes) dominate
CML=More immature(promyelocyte and myelocyte)
absolute basophilia?
LR=not present
CML=present
sign and symptom of CML?
blast stage
accelerated stage
chronic stage
blast stage?
symptoms are most likely fever, bone pain, and an increase in bone marrow fibrosis
accelerated stage?
Some (<10%) are diagnosed in this stage bleeding petechiae ecchymosis In these patients, fevers are most commonly the result of opportunistic infections
chronic?
Most patients (~90%) are diagnosed during this stage which is most often asymptomatic.
diagnosed incidentally with an elevated white blood cell count on a routine laboratory test.
enlarged spleen and liver and the resulting upper quadrant pain this causes.
The enlarged spleen may put pressure on the stomach causing a loss of appetite and resulting in weight loss.
It may also present with mild fever and night sweats due to an elevated basal level of metabolism
Risk factor?
age of 65 years
Exposure to ionizing radiation ( about 10 years after the exposure
