Diagnostic criteria of antiphospholipid antibody syndrome
- clinical + 1 lab must be met
lab: lupus anticoagulant, anticardiolipin antibody, anti-beta-2 gp antibody - clinical: arterial or venous vascul thromb, 3 or more consecutive unexpl fetal loses before 10 week, 1 unexplained fetal loss oafter 10 weeks, 1 premature birth of normal neonate befoere 14 week (due to preexlampsia, eclampsia, placental insuf)
lupus anticoagulant - PTT
prolonged PTT in vitro –> it will not corect if mixed in a 1:1 dilutionwith normal plasma
lupus anticoagulant test
- Russel viper venom test
2. kaolin cloating time
polycytemia vera - treatment
phlobotomy
hydroxyurea –. decreases thrombus
polycythemia vera - ESR
decreased
CLL - lymph node biopsy vs BM biopsy
biopsy is not needed
CLL - diagnostic test
- smagde cells on smear
- flow cytometry
- lymph node or BM biopsy not needed
possibility of DVT –> ….. (next step)
compression U/S
PE vs DVT on the initiation of anticoagulant
start in PE
confirm with U/S first in DVT
epidural spinal cord compression 2ry to cancer - management / MC locations / pain worse at
first steroids –> then MRI –> then maybe radiation + neurosurgery consultation
MC locations: 60% thoracic, 30% lumbosacral
- pain worse at night at recumbent position
waldestrom vs MM regarding BM biopsy
MM: more than 10% clonal plasma cells
Wald: more than 10% clonal B cell
waldestrom major complciations
lymphadenopathy, HEPATSPLENOMEGALY, bleeding, neuropathy, hyperviscosity
2 things unexplain on waldestrom
lymphadenopathy, hepatosplenomegaly
how to confirm HIT 2
- Elisa for PF4 antibodies
2. Seretonin release assay
G6PD def - normal G6PD activ level?
may be normal during attack
in which leukemia, PCP prophylaxis is indicated
CLL
Contraidications of thrombolytics in a massive PE –>
consider mechenical thrombectomy
iliac stenting
surgical thrombectomy
contraidications of anticoagulation in PE/DVT –> …
IVC filter
Risk factor for PE from thrombus in the renal vein
nephrotic syndrom
SVC - next step
X-ray
1st choice for cancer related vomiting
5-HT3 antagonists
MCC of folate def in USA
alcohol
MCC of inadequate response to EPO in patients with renal failure
iron def
Leukemoid reaction vs CML regarding leukocyte count and LAP score
leukemoid: more than 50, high LAP
CML usually more than 100, low LAP
leukemoid reaction vs CML regarding type of cells
leukemoid reaction: more mature: metamyelocytes more than myelocytes / no basophilia
CML: less mature: metamyelo less than myelocytes, absolute basophilia
ITP - treatment
A. children: skin manifesatation: observe, bleeding: IVIg OR glucocorticoids
B. Adults: if platelets less than 30 without bleeding: observe, if platelets less than 30 or bleeding: IVIg OR glucocorticoids
hyperspleenism - platelets number
usually more than 30 –> no bleeding
other tests required for ITP
HIV + HCV
how to treat anemia of chronic disease
treat the underlying condition (for example for RA, give methotrexate - not EPO)
iron is not beneficial
hered spheorcytosis with pain at RUQ?
acute cholocystitis
treatment of acute PE or DVT - oral factor Xa inh (abixaban rivaroxaban) vs warfarin
- oral factor Xa inh has 2-4 hours time of onset and they do not need overal or Lab monitorin
- Warfarin has 5-7 days onset, need overlap and lab monitorin (PT/INR)
PE/DVT - thrombolysis?
only for PE with hemodynamic instability or (rarely) for massive proxima DVT with symptoms and ischemia
Trousseau’s sign?
hypercoagulability disorder presenting with recurent superficial thrombophlebitis at unusual sites (eg. arms, chest area). It is usually associated with occult visceral malignancy such as pancreatic (MC), stomach, lung, or prostate carcinoma
when to do extensive testing for an underlying inerited cause of thrombosis
when patient’s history is suggestive:
1. yougner than 45 2. Reccurent DVT 3. multiple or unusual sites 4. Family history
Bone scan for the diagnosis of MM
not useful: detects only osteoblastic activity (metastatic tumores)
screening test for MM
- serum protein electrophoresis
- urine protein electrophoresis
- free light chain analysis
prostate cancer in a patient with bone metastasis with history of orchiectomy
radiation (NO FLUTAMIDE)
how to diagnose autoimmune hemolytic anemia
coomb: positive for anti-IgG, anti-C3
coomb: positive for IgM, anti-C3
IgM anemia - treatment
avoidance of cold
rituximab +/- fludarabine
autoimmune hemolytic anemia - complications
IgG: venous thromboembolisms, lymphoproliferative disorder
IgM: ischemia, periphearl gangrene, lymphoproliferative disorder
sideroblastic anemia - size
both normal and micro
osteoarthritis - anemia of chronic disease
NO –> it is not a systemic infl condition
before preganncy test - suspicion for anemia in the family - initial tests?
african: Hb elctrophoresis + CBC
non african: CBC at the beginning
salvage therapy?
treatment when standard therapy fails
neoaduvant therapy?
treatment given before standard therapy
adjuvant therapy ?
therapy given in addition to standard therapy
induction therapy
initial dose of treatment to rapidly kill tumor cells and send the patient into remission
consolidation therapy?
after induction therapy with multidrug regimens to further reduce tumore burden
maintenance therapy?
after induction and consolidation therapies (or initial standard therapy) to kill any residual tumor cells and keep the patient in remission (eg. daily androgen in prostate cancer)
Hair cell leukemia - genetics / hepatomegaly? / lymphadenopathy / prognosis
BRAF mutation
hepatomegaly + lymphadenopathy are both rare
life expectancy is near normal
hair cell leukemia - number of WBC
decreased due to BM inflitration
a minority has mild leukocytosis
Blood transfusion reactions associated with hypotension (and onset and explain)
- anaphylactic: sec to minutes
- sepsis: minutes to hours
- primary hypotension reaction: transient, in patients with ACEi: within mins (by bradykynin)
- tranfusion related acute lung injury: noncardogenic pul edema: within 6 hours: causef by donor anti-leukocytes antibodies against recipient neutrophils + pulm endothelial cells
indications for washed blood trasfusion
- IgA def
- Complement-dependet autoimmune hemolysis
- Continued allergic reactions (e.g. hives) with red cell transfusion despite antihistamine treatment)
indications for irradiated blood transfusion
- BM trannplant recipients
- Acquired or congenital cellular immunodef
- Blood components donated by 1st or 2nd degree relatives
indications for leukoreduced blood transfusion
- Chronically transfused patients
- CMV negative at risk patients (eg. AIDS, transplants patients)
- potential transplant recipients
- previous febrile nonhemolytic reaction
MM - renal insuficiency at the time of diagnosis?
in 50%
MM - protein gap
elevated (more than 4): it is the difference between total protein and albumin
what is needed in the metabolism of homocysteine to methionine
- folate
- B12
- B6
HIT 1
nonimmune direct effect of heparin on platelet activation and usually presents within the 1st 2 days of heparin –> platelet then normalises with continued heparin therapy and there are no clinical consequences
Patients with asplenia are at risk for fulimnant infection with encapsulated bacteia due to deficits in
antibody response and antibody-mediated pahgocytosis/complement activation
cannabinoids in anorexia?
only HIV anorexia
NOT cancer related
cancer related anoreia/cachexia syndrome - treatment
progesterone analogues (megestrol acetate + medroxyprogesterone acetate) and corticosterois (progesterone are preferred due to less SEs)
best initial test after suspicion of SCC of head and neck
panendoscopy (triple endoscopy: esophagoscopy, bronchoscopy, laryngoscopy)
β symptoms in AML
only fatigue is common
the liver can store vit D for …. (time)
30 days
but an acutely ill person with underlying liver disease can become K def in 10 days
transplantation graft types and definitions
- autograft –> from self
- synergic graft (isograft) –> from identical twin or clone
- allograft (homograft) –> from nonidentical individual of same species
- xenograft –> from different species
transplant rejection - types and onset (time)
- hyperacute –> minutes
- acute –> weeks to months
- chronic –> months to years
- graft-vs-host disease –> varies
hyperacute transplant rejection - result onset / pathogenesis / treatment
widespread thrombosis of graft vessels –> ischemia/necrosis
onset: within minutes
Pre-existing recipient antibodies react to donor antigen and activate complement (type II hypersensitivity
- graft must be removed
acute transplant rejection - mechanism
- Cellular: CD8 T cells activated against donor MHCs (type IV hypersensitivity reaction)
- Humoral: antibodies formation (after transplant) –> activate complement
acute transplant rejection - results in / onset
vasculitis of graft vessels with dense interstitial lymphocytes infiltrate
onset: weeks to months
acute transplant rejection - management
prevent/reverse with immunosuppresant
chronic transplant rejection - mechanism
CD4 T cells respond to recipient APCs presentinf donor peptides, including allogenic MCR –> cytokines –> cellular and homural compoments (type II and IV hypersensitivity reactions)
chronic transplant rejection - results in / onset
cytokines –> proliferation of vascular SMCs, paremchymal atrophy, Interstitial fibrosis
DOMINATED BY ARTERIOSCLEROSIS
onset: month years
chronic transplant rejection - example of organs and results
- lung –> bronchiolitis obliterans
- Heart –> accelerated atherosclerosis
- Kidney –> chronic graft nephropathy
- Liver –> vanishing bile duct syndrome
Graft-versus-host-disease - Mechanism / onset
grafted immunocompompetent T cells proliferate in the immunocompromised host and reject host cells with “foreign” proteins –> severe organ dysfunction (type IV hypersensitivity reaction)
onset: varies
Graft-versus-host-disease - requirements to occur
- the graft must contain immunocommpoment T cells
- the host must be immunocompromised
- the recipient must express antigens (eg. MHC or other minor proteins) foreign to the donor
Graft-versus-host-disease - clinical manifestation
- maculopapular rash
- jaundice
- diarrhea
- hepatsplenomegaly
Graft versus tumor effect - definition
Donor T-cells eliminate malignant residual host T-cells (graft versus leukemia) or eliminates diverse kinds of tumors.
transplant rejection - types, onset (time) and hypersensitvity reaction
- hyperacute –> minutes –> type 2
- acute –> weeks to months –> type II (humoral) or IV (cellular)
- chronic –> months to years –> both IV and IV (humoral and cellular)
- graft-vs-host disease –> varies –> type IV
HIT 2 - management
stop all heparin products
- start direct thrombin inhibitor (argatroban) or fondaparunix
HIT 2 clinical features
suspected with heparin exposure more than 5 days + any of the following
- platelet reduction more than 50%
- arterial or venus thrmbosis
- necrotic skin lesions at heparin injection sites
- acute systemic (anaphylactoid) reactions after heparin)
patients with asplenia are at risk for fulminant infection with encapsulated bacteria due to deficits in
antibody response and antibody-mediated phagocytosis/complement activation
how to confirm MM
bone marrow biopsy: more than 10% clonal plasma cells
TTP management
- plasma exchange
- glucocorticoids
- rituximab
osler weber rendu sundrome - mechanism of polycythemia
shunts in the lung
vitamin K - store
30 days
- in acutely ill with underlying liver disease: 7-10 d
delayed hemolytic blood transfusion reaction
- mild fever + hemol anema
- within 2-10 days
- (+) direct coombs
- by anamnestic antibody response
in the absence of any clear provoking factors (recent procedure, immobilization etc), patients with 1st episode of venous thromboembolism should
be referred for age appropriate cancer screening
CO poisoning
high flow 100% O2
- intubation/hyperbaric O2 therapy (sever)
giant cell bone tumor - area of the bone
epiphysis
heparin vs LMWH - PTT
heparin it is more likley to prolong it
CLL - worse prognosis if
- hepatosplenomegaly
- anemia + thrombocytopenia
- multiple chain lymphadenopathy
wafarin induced skin necrosis in protein C def - management
immediate warfarin cessation and administration of protein C concentrate
MM - immunodef
bone marrow infiltration by neoplastic cells –> alters normal leukocyte population and causes hypogammaglobulinemia
MC mechanism of pancytopenia in SLE
peripheral destruction
symptomatic patients with autoimmune hemolytic anemia - treatment
high dose steroids
methionine level in megaloblastic anemia
decreased
immunologic transfusion reaction types (MC?)
- allergic reaction
- Anaphylactic reaction
- Febrile nonhemolytic transfusion reaction (MC)
- Acute hemolytic tranfusion reaction
- transfusion related acute lung injury
- delayed hemolytic
tranfusion related acute lung injury
noncardogenic pul edema
within 6 hours
caused by donor anti-leukocytes antibodies against recipient neutrophils + pulm endothelial cells
delayed hemolytic blood transfusion reaction
- mild fever + hemol anema
- within 2-10 days
- (+) direct coombs
- by anamnestic antibody response
febrile nonhemolytic reaction
caused by cytokines accumulation during blood storage
- 1-6 hours
- fever + cilld
Blood transfusion reaction - acute hemolytic transfusion reaction - intravascular or extravascular hemolysis?
both:
hemoglobinuria (intrav hemolysis)
jaundice (extravascular hemolysis)
Blood transfusion reaction - acute hemolytic transfusion reaction - mechanism
Type II hypersensitivity reaction –> intravascular hemolysis (ABO incompatibility) or extravascular hemolysis (host antibody reaction against foreign antigen on donor RBCs)