-
what microcytic anemia has low RDW/?
Iron deficiency
-
Best initial test for microcytic anemia?
ferritin
-
Who gets IM/IV iron?
person that refuses blood transfusion
-
What can cause anemia and neurological symptoms?
- alcohol,
- b12
- folate deficiencies
-
How do you become deficient in folate?
- increased cell turnover is most common
- decreased leafy intake is 2nd most common
-
Why do you order methylmalonyl acid level?
bc b12 levels could be normal but you are truly deficient
-
What is the smear with sickle cell trait vs disease?
- trait--normal
- disease-- sickled cells
-
the only clinical manifestation of sicle cell trait?
renal
-
sickle cell + fever, next best step'?
antibiotics
-
large Drop in ht in siclke cell patient?
parvo b 19 virus
-
Most accurate test for parvo b19?
-
best treatment for parvo19 ?
iv immunoglobulin
-
treatment for priapism, cns, eye infarctions in sickle cell?
exchange transfusion
-
How do you prophylax against sickle cell crisis?
hydroxyurea
-
RX for autoimmune anemia, hereditary spherocytosis?
splenectomy
-
-
What anemia has heinz bodies?
G6PD deficiency causing anemia
-
-
What is the most common oxidant stress?
infection
-
Which type of Acute leukemia has auer rods and myeloperoxidase?
CALLA +?
-
Which AML presents with DIC?
M3
-
All forms of AML except for m3 are treated with?
-
M3 of AML is treated with?
retinoic acid
-
Treatement for ALL?
- doxorubicin
- vincristine
- prednisone
-
Rx for relapsed acute leukemia?
BMT
-
Who should get transplanted after 1st remission?
bad cytogenetics
-
ALL relapses where?
RX?
- in the spinal chord
- Intrathecal MTX
-
When are xenografts used?
heart valves
-
4 oncologic emergenices?
- hypercalcemia
- superior vena cava syndrome
- leukostasis
- spinal cord compression
-
What WBC count increases risk for leukostasis?
100,000
-
HOw do you treat leukostasis?
leukopheresis
-
in What age groups are ALL and AML present?
-
Presence of what chromosome in ALL shows poor prognosis?
9:22
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Which leukemia has smudge cells?
CLL
-
What is the survival expectancy for acute vs chronic leukemias?
- acute--6 to 12 weeks from diagnosis
- chronic-6 to 12 years from diagnosis
-
MCC side effect of CLL?
autoimmune hemolytic anemia
-
Leukemia vs leukemoid?
- LAP--high---leukemoid rxn
- low--leukemia
-
most common cause of death in cml?
blast crisis
-
Rx for CML?
imatinib--bcr-abl chromosome antagonist
-
-
underage what age do u do allogenic vs autologous bone marrow transplant?
- allogenic- less than 50
- autologous less than 70
-
neoplasm associated with down syndrome?
ALL
-
neoplasm associated with plummer vinson syndrom--atrophic glossitis, esophageal webs, anemia
squamous cell carcinoma
-
neoplasm associated with xeroderma pigmentosum, albinism?
- melanoma
- basal cell ca
- squamous cell ca
-
neoplasm associated with tuberous sclerosis
- astrocytoma
- angiomyolipoma
- cardiac rhabdomyosarcoma
-
neoplasm associated with chronic atrophic gastritis, pernicous anemia, post surgical remnants?
gastric adenocarcinoma
-
neoplasm associated with actinic keratosis?
squamous cell
-
neoplasm associated with barrets esophagus?
esophageal adenocarcinoma
-
neoplasm associated with cirrhosis?
HCC
-
neoplasm associated with ulcerative colitis?
colonic adenocarcinoma
-
neoplasm associated with pagets disease of bone?
- secondary osteosarcoma
- fibrosarcoma
-
neoplasm associated with immunodeficiency?
malignant lymphomas
-
neoplasm associated with Aids
- kaposi sarcoma
- non hodkings lymphoma
-
Causes of Microcytic anemia?
- Thalasemia
- Iron deficiency
- Chronic disease
- sideroblastic anemia--ala synthatase def
-
+ dipstick for blood can be what 3 entities?
-
Nephrotoxic drugs?
- amphotericin
- aminoglycoside
- acyglovir
- cyclosporine
- cisplatin
-
Which form of thalasemia has increased reticulocyte count?
3 gene deletion or hemoglobin H
-
Thalassemia beta minor and major both have what hemoglobin?
fetal
-
How is thalassemia beta minor diagnosed on electrophoresis?
hbA2 >3.5 %
-
5 conditions that lead to cessation of heme synthesis?
- ala synthase-- sideroblastic anemia/etoh/inh
- ala dyhydratase/ferrochelatase- lead
- porphobilinogen deaminase-- acute intermittent porphyria
- uroporphyrinogen decarboxylase- cuteanea porphyria tarda
-
what inhibits ala synthase?
glucose and heme
-
liver disease, def of b12 and folate cause macrocytic anemia but what 2 only cause hypersegmented neutrophils?
b12 or folate deficiency
-
after suspective a macrocytic anemia and cbc showing large mcv, what is the next best step in management?
peripheral smear to see the presence of hypersegmented neutrophils
-
Best initial test in suspecting anemia?
CBC
-
Best initial test in suspecting SS?
Best initial test in suspecting macrocytic anemia?
peripheral smear
-
Most accurate test in suspecting ss?
electrophoresis
-
Most common presentation for children with SS?
dactylitis
-
What is seen in Ehrlichia infections?
morulae inside neutrophils
-
SS with fever or high WBC, best next management?
antibiotics
-
When is exchange transfusion used in SIckle cell?
- severe vasoocclusive crisis:
- acute chest syndrome
- priapism
- stroke
- retinal infarction
-
BEst initial test in a hct drop in a sickle cell pt?
reticulocyte count due to possible parvo b19 infection
-
What is the only defect in sickle cell trait?
cant concentrate the urine
-
most accurate test for hereditary spherocytosis?
osmotic fragility
-
most accurate test for PNH?
hams test
-
What causes autoimmune hemolysis?
- CLL
- lymphoma
- sle
- alphamethyl dopa
-
most accurate diagonistic test for autoimmne hemolytic anemia?
coombs test
-
autoimmune hemolytic anemia can be igg or igm?
what causes each?
- igg--cll, sle, lymphoma, alphamethyl dopa
- igm--mycoplasma, infections mono, infections
-
What 2 causes of anemia give spherocytes?
- hereditary spherocytosis
- autoimmune hemolysis when ig pinch of a part of the membrane and the rbc becomes a sphere instead of biconcave shape
-
best intitial therapy for ITP and autoimmune hemolytic anemia?
steroids
-
what rx is for recurrent autoimmune hemolysis and ITP?
splenectomy
-
severe, acute hemolysis or thrombocytopenia is best treated with ?
IV Immunoglobulins
-
MOA of cyclophosphamide?
covalent linking of DNA at guanine N-7
-
Toxicity of cyclophosphamide and ifofsamide?
hemorrhagic cystitis, prevent with mesna
-
MOA of azathioprine?
antimetabolite precursor to 6-MP
-
What drug increases toxicity of Azathioprine?
allopurinol
-
Alternative treatments to steroids in autoimmune disease like hemolysis?
- cyclophosphamide
- cyclosporine
- azathioprine
- mycophenolate mofetil
-
Rx for autoimmune hemolytic anemia when splenectomy doesnt help?
Rituximab
-
Rx for cold agglutinins vs warm agglutinins?
- cold: Warm:
- rituximab steroids
- splenectomy
- immunosuppresants
- cyclophosphamide, cyclosporine, azathioprine
-
What is similar between cryoglobulins and cold agglutinins?
Differences?
- both are igM and dont respond to steroids
- cryoglobulins are seen with hep c, causes joint pain and glomerulonephritis
-
What is the best initial test for G6PD deficiency?
MOst accurate test?
- heinz bodies and bite cells on smear
- G6PD level 2 months after a hemolytic event
-
HUS and TTP are both treated wtih?
plasmapheresis
-
What is the most accurate test for PNH?
decreased levels of cd55/59
-
What is the best initial therapy for PNH?
steroids
-
What inactivates c5 in the complement pathway and improves PNH?
Eculizumab
-
Next best step when there is delay to plasmapharesis?
transfuse ffp
-
How is a true aplastic anemia due to autoimmune destruction of the bone marrow by Tcells treated?
what if pt is over 50 or there is no matched donor?
- by an allogenic bone marrow transplant
- anti-thymocyte globulin or cyclosporine/tacrolimus
-
mnemonic for DDx?
- acquired
- vascular
- inflammatory
- trauma/toxin
- autoimmune
- metabolic/medications
- Infection
- Neoplastic
- congenital
- Degenerative
- Endocrine/electrical
-
Most accurate test for Polycythemia Vera?
JAK2 mutation
-
What mutation is seen in chronic myeloproliferative disorders such as polycythemia vera, essential thrombocytosis and myelofibrosis?
JAK2 mutation
-
WHat kind of kidney pathology increases epo?
any--cyst, hydronephrosis, wilms tumor, RCC
-
The best initial therapy for essential Thrombocytosis is ?
hydroxyurea
-
The best initial therapy for Polycythemia vera and Essential Thrombosis is what?
Hydroxyurea
-
Painfull red hands in Essential thrombosis are treated with?
aspirin
-
The best initial test in AML is?
peripheral smear
-
What is the most accurate test in AML?
flow cytometry
-
What is the translocation in AML M3?
between chr 15 and chr 17
-
MPO and Auer Rods are characteristic of what leukemia?
AML
-
What chemotherapy does ALL require?
intrathecal therapy for CNS
-
What is the next best step after a high neutrophil count is found in CML?
Leukocyte alkaline phosphatase level
-
MOst accurate test in CML?
bcr-abl pcr
-
What is the rx for leukostasis?
plasmapharesis
-
What kind of characteristic deletion is seen in MDS?
5q deletion
-
extreme friendliness, elfin facies with deletion of ch 7 is what syndrome?
WIlliams
-
Bilobed nucleus found in MDS is also called?
Pelger-Huet Cells
-
conversion of CLL into high grade lymphoma is called?
Richter Phenomenon
-
What myeloproliferative disorders most commonly converts into AML?
CML
-
Best initial for CML?
Most effective cure for CML?
-
What is done for CLL stage 0 and 1?
2,3,4?
- nothing
- fludarabine + rituximab
-
Refractory cases of CLL are treated with?
Mild Cases?
severe infections?
ITP or AIHA?
- cyclophosphamide
- Chlorambucil
- steroids
-
Most accurate test for Hairy cell leukemia?
TRAP or CD11c
-
Best initial Therapy for NHL?
excisional biopsy
-
What are the staging procedures for NHL?
- CT of chest, abdomen, pelvis
- BOne marrow biopsy
-
What is meant by the four stages for CLL?
1,2,3,4
- 0--high Wbc
- 1-lymphadenopathy
- 2- hepatosplenomegaly
- 3-anemia
- 4- thrombocytopenia
-
When is local radiation done in NHL?
stage 1a and IIa
-
How is Stage III and IV or any condition with B symptoms treated?
- Cyclophosphamide
- Hydroxydoxorubicin
- Oncovorin- vincristine
- Prednisone
- Rituximab
-
Rx for hairy cell leukemia?
cladribine
-
PCP prophylaxis is indicated for which leukemia?
CLL
-
What is the causative organism in MALT?
- H. pylori--treat with
- ppI
- metro
- tetracycline
- bismuth
-
How are hodkin and NHL treated?
- both in stages Ia and IIa get radiation
- III and IV--chemotherapy--- adriamycin
- bleomycin
- vincristine
- dacarbazine
-
Staging for NHL and HD?
- stage 1- 1 lymph node group
- stage 2- 2 or more lymph node groups on one side of diaphragm
- stage 3- both sides of diaphragm
- stage 4- widespread disease
-
How are relapses after radiation therapy treated in NHL and HD?
after chemotherapy?
- with chemotherapy
- extra high doses of chemotherapy
-
calcemia
renal failure
anemia
bone lesions lytic
MM
-
Best initial test in suspecting MM?
Xray
-
What is the most accurate test for MM?
bone marrow biopsy showing more than 10% plasma cells
-
What does a technetium bone scan pick up?
osteoblastic activity such as in a prostate ca
-
BEST INITIal therapy for MM?
- dexamethasone
- lenalidomide
- bortezomib
-
Hypercalcemia leads to what kind of symptoms?
- stones- renal
- bones--lytic lesions bones
- groans-constitpation
- moans--psychotic
-
What monoclonal gammopathy produces hyperviscosity?
waldernstrons gammopathy
-
Best intital therapy for Waldernstrons gammaglobulienemia?
plasmapharesis
-
What are dutcher bodies in waldenstrons gammaglobulinemia?
pas+ IGM around the nucleus
-
Management of severe bleeding due to thrombocytopenia?
IViG
-
next best step when splenectomy or steroids arent effective in regulating ITP?
- rituximab
- azathioprine
- cyclosporine
- mycophenolate
-
best initial therapy for VWF dz?
- desmopressin
- then factor 8
-
most accurate test for hemophilia
specific assay for the missing factor 8 or 9
-
factor 11 or 12 deficiency has increased bleeding/?
factor 11
-
What replaces fibrinogen in DIC?
cryoprecipitate
-
cause of hypercoagulabitliy
- factor V leiden
- ATIII deficiency
- Prothrombin mutation
- protein C and S
-
Tests to confirm HIT?
serotonin release assay
platelet factor 4 antibody elisa
-
Rx for HIT??
lepirudin, bivalirudin direct thrombin inhibitor
-
what is the only cause of thrombosis that elevates PTT?
antiphospholipid syndrome
-
BEst initial test for APL is ?
most accurate test for lupus anticoagulant is ?
- mixing study
- russel viper venom test
-
what anemia can progress to acute leukemia?
sideroblastic anemia
-
cause of hydrops fetalis?
hemoglobin bart-- 4 gamma chains
-
hemoglobin a2?
2 delta chains
-
what causes sickling of sickle cells?
- hypoxia
- infection
- dehydration
- acidosis
-
on what antibiotic are sickle cell pts on untill 5 years old?
penicillin
-
acanthocyte (spurr or spiky) is seen where?
abetalipoproteinemia
-
How do steroids affect wbc count?
- raises it acutely
- but diminishes it chronically
-
Causes of eosinophilia?
- drugs
- neoplasm
- atopic
- asthma
- adrenal insuffiecncy
- ain
- autoimmune
- collagen vascular disease
- parasites
-
how u monitor lmwh?
antifactor Xa
-
symptoms of polycythemia vera?
- thrombosis
- eythromelalgia- burning in the hands and feet
- pruritis
-
Rx for pv?
- aspirin for thrombosis and for the erythromelalgia
- antimetabolite
-
most common form of hodking disase?
nodular scleroris--fibrosis of l. nodes
-
horshoe kidney, short stature, varioius pigmentation, pancytopenia, absence of thumbs
fanconi anemia
-
What is the WAGR complex?
- wilms tumor
- aniridia
- genitourinary malformation
- retardation--mental-motor
-
-
causes of basophilic stapling?
-
vit b12 def vs folate def?
no neurological symp;toms in folate deficiency
-
what anemia goes on to acute leukemia in 10% of patients?
sideroblastic anemia
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