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what microcytic anemia has low RDW/?
Best initial test for microcytic anemia?
Who gets IM/IV iron?
person that refuses blood transfusion
What can cause anemia and neurological symptoms?
- 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?
- disease-- sickled cells
the only clinical manifestation of sicle cell trait?
sickle cell + fever, next best step'?
large Drop in ht in siclke cell patient?
parvo b 19 virus
Most accurate test for parvo b19?
best treatment for parvo19 ?
treatment for priapism, cns, eye infarctions in sickle cell?
How do you prophylax against sickle cell crisis?
RX for autoimmune anemia, hereditary spherocytosis?
What anemia has heinz bodies?
G6PD deficiency causing anemia
What is the most common oxidant stress?
Which type of Acute leukemia has auer rods and myeloperoxidase?
Which AML presents with DIC?
All forms of AML except for m3 are treated with?
M3 of AML is treated with?
Treatement for ALL?
Rx for relapsed acute leukemia?
Who should get transplanted after 1st remission?
ALL relapses where?
- in the spinal chord
- Intrathecal MTX
When are xenografts used?
4 oncologic emergenices?
- superior vena cava syndrome
- spinal cord compression
What WBC count increases risk for leukostasis?
HOw do you treat leukostasis?
in What age groups are ALL and AML present?
Presence of what chromosome in ALL shows poor prognosis?
Which leukemia has smudge cells?
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
most common cause of death in cml?
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?
neoplasm associated with plummer vinson syndrom--atrophic glossitis, esophageal webs, anemia
squamous cell carcinoma
neoplasm associated with xeroderma pigmentosum, albinism?
- basal cell ca
- squamous cell ca
neoplasm associated with tuberous sclerosis
- cardiac rhabdomyosarcoma
neoplasm associated with chronic atrophic gastritis, pernicous anemia, post surgical remnants?
neoplasm associated with actinic keratosis?
neoplasm associated with barrets esophagus?
neoplasm associated with cirrhosis?
neoplasm associated with ulcerative colitis?
neoplasm associated with pagets disease of bone?
- secondary osteosarcoma
neoplasm associated with immunodeficiency?
neoplasm associated with Aids
- kaposi sarcoma
- non hodkings lymphoma
Causes of Microcytic anemia?
- Iron deficiency
- Chronic disease
- sideroblastic anemia--ala synthatase def
+ dipstick for blood can be what 3 entities?
Which form of thalasemia has increased reticulocyte count?
3 gene deletion or hemoglobin H
Thalassemia beta minor and major both have what hemoglobin?
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?
Best initial test in suspecting SS?
Best initial test in suspecting macrocytic anemia?
Most accurate test in suspecting ss?
Most common presentation for children with SS?
What is seen in Ehrlichia infections?
morulae inside neutrophils
SS with fever or high WBC, best next management?
When is exchange transfusion used in SIckle cell?
- severe vasoocclusive crisis:
- acute chest syndrome
- 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?
most accurate test for PNH?
What causes autoimmune hemolysis?
- alphamethyl dopa
most accurate diagonistic test for autoimmne hemolytic anemia?
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?
what rx is for recurrent autoimmune hemolysis and ITP?
severe, acute hemolysis or thrombocytopenia is best treated with ?
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?
Alternative treatments to steroids in autoimmune disease like hemolysis?
- mycophenolate mofetil
Rx for autoimmune hemolytic anemia when splenectomy doesnt help?
Rx for cold agglutinins vs warm agglutinins?
- cold: Warm:
- rituximab steroids
- cyclophosphamide, cyclosporine, azathioprine
What is similar between cryoglobulins and cold agglutinins?
- 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?
What is the most accurate test for PNH?
decreased levels of cd55/59
What is the best initial therapy for PNH?
What inactivates c5 in the complement pathway and improves PNH?
Next best step when there is delay to plasmapharesis?
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?
Most accurate test for Polycythemia Vera?
What mutation is seen in chronic myeloproliferative disorders such as polycythemia vera, essential thrombocytosis and myelofibrosis?
WHat kind of kidney pathology increases epo?
any--cyst, hydronephrosis, wilms tumor, RCC
The best initial therapy for essential Thrombocytosis is ?
The best initial therapy for Polycythemia vera and Essential Thrombosis is what?
Painfull red hands in Essential thrombosis are treated with?
The best initial test in AML is?
What is the most accurate test in AML?
What is the translocation in AML M3?
between chr 15 and chr 17
MPO and Auer Rods are characteristic of what leukemia?
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?
What is the rx for leukostasis?
What kind of characteristic deletion is seen in MDS?
extreme friendliness, elfin facies with deletion of ch 7 is what syndrome?
Bilobed nucleus found in MDS is also called?
conversion of CLL into high grade lymphoma is called?
What myeloproliferative disorders most commonly converts into AML?
Best initial for CML?
Most effective cure for CML?
What is done for CLL stage 0 and 1?
- fludarabine + rituximab
Refractory cases of CLL are treated with?
ITP or AIHA?
Most accurate test for Hairy cell leukemia?
TRAP or CD11c
Best initial Therapy for NHL?
What are the staging procedures for NHL?
- CT of chest, abdomen, pelvis
- BOne marrow biopsy
What is meant by the four stages for CLL?
- 0--high Wbc
- 2- hepatosplenomegaly
- 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?
- Oncovorin- vincristine
Rx for hairy cell leukemia?
PCP prophylaxis is indicated for which leukemia?
What is the causative organism in MALT?
- H. pylori--treat with
How are hodkin and NHL treated?
- both in stages Ia and IIa get radiation
- III and IV--chemotherapy--- adriamycin
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?
- with chemotherapy
- extra high doses of chemotherapy
bone lesions lytic
Best initial test in suspecting MM?
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?
Hypercalcemia leads to what kind of symptoms?
- stones- renal
- bones--lytic lesions bones
What monoclonal gammopathy produces hyperviscosity?
Best intital therapy for Waldernstrons gammaglobulienemia?
What are dutcher bodies in waldenstrons gammaglobulinemia?
pas+ IGM around the nucleus
Management of severe bleeding due to thrombocytopenia?
next best step when splenectomy or steroids arent effective in regulating ITP?
best initial therapy for VWF dz?
- 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/?
What replaces fibrinogen in DIC?
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?
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?
cause of hydrops fetalis?
hemoglobin bart-- 4 gamma chains
2 delta chains
what causes sickling of sickle cells?
on what antibiotic are sickle cell pts on untill 5 years old?
acanthocyte (spurr or spiky) is seen where?
How do steroids affect wbc count?
- raises it acutely
- but diminishes it chronically
Causes of eosinophilia?
- adrenal insuffiecncy
- collagen vascular disease
how u monitor lmwh?
symptoms of polycythemia vera?
- eythromelalgia- burning in the hands and feet
Rx for pv?
- aspirin for thrombosis and for the erythromelalgia
most common form of hodking disase?
nodular scleroris--fibrosis of l. nodes
horshoe kidney, short stature, varioius pigmentation, pancytopenia, absence of thumbs
What is the WAGR complex?
- wilms tumor
- genitourinary malformation
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?
What would you like to do?
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