HemeOnc Flashcards
High TIBC
Iron deficiency
> 24oz/d cow’s milk + anemia, pica, fatigue, inc RDW
Iron deficiency –> oral iron therapy
Iron deficiency tx in baby
Cows milk b4 9mo
High Ferritin
Chronic disease –> EPO
Low iron, Low TIBC anemia
Chronic disease
- high ferritin
Low TIBC
Everything else normal or high
Thalessemias (Inc A1)
High Hb A2
B-Thal trait
MCV <75, normal RDW, Target cells, Normal iron, ferritin, no response to iron
B-Thal trait (inc A2), alpha-thal (normal)
Target cells anemia
Thal minor
Hct >30, RDW NORMAL, RBC count normal to Inc, normal to inc iron, Ferritin, anemia
Thal minor
Basophilic stippling
Thalessemia & heavy metal poisoning
RUQ pain w/ inspiration, splenomegaly, same thing in brother
Hereditary spherocytosis –> Ca-bilirubin gall stones –> folate & splenectomy
Tx for hereditary spherocytosis
Folate & tranfusions –> 30+ yr strep risk = vaccination b4 splenectomy + daily PCN
Multiple small round dense, hyperchromic (no pallor) RBCs, RUQ pain, Coombs -
Hereditary spherocytosis = ankyrin defect –> high MCHC & RDW
High retic, hyperUNconjugated bilirubinemia, spherocytes, schistiocytes, osmotic fragility
Hereditary spherocytosis
High retic, inc MCHC, normal or low MVC, anemia, jaundice
Hereditary spherocytosis
Thrombocytopenia –> pancytopenia, absent radius/thumb, café-au-lait, microcephaly
Fanconi anemia (TAR)
Pancytopenia, hypopigmented spots, short, abn thumbs, deafness, strabismus, 7 y/o
Fanconi anemia = DNA breaks –> stem cell transplant
Swelling of hand/feet, fever, pale, poor feeding
Hand-foot syndrome = Sickle Cell
85% HbS, 15% HbF
Sickle cell disease
Management of Sickle cell
Vaccinations, PCN until 5, folic acid, HU for vasoocclusive events
Sickle cell disease complications
ACS, dacylitis, stroke, osteomyelitis, splenic infarction
Hx severe arm pain, now weakness, clumsy in right hand, 3 y/o A.A boy
Sickle cell stroke = get peripheral smear, retic
40%HbS, 60% HbA
Sickle cell Trait –> Hematuria, can’t concentrate urine –> polyuria, nocturia, inc UTIs
Anemia + high retic, A.A boy, hx hematuria, Abd pain, no vaccines –> death
Sickle cell –> bacterial infection d/t asplenia = PCN until 5 y/o
1 bacteremia bug in sickle cell
Strep pneumo (encapsulated)
Howell-Jolly bodies
Sickle cell - sign of aplenia
Severe drop in Hb, retic <1%, sudden onset fatigue, dyspnea, NO HSM
Aplastic crisis
Retic <1%, BM HYPOCELLULAR, fatty infiltrate, bleeding, petechiae, lethargy, weakness
Acquired aplastic anemia - drugs, chemicals, CCl4, insecticides
Hypoplastic, fat-filled marrow
Aplastic Anemia
High F Hb (HU increases this)
Sickle cell, beta thal, aplastic anemia
Reticulocytosis w/ sickle cell
Hyperhemolytic crisis
African American 17 y/o male w/ painless hematuria
Sickle cell trait - d/t papillary ischemia
Heinz bodies & bite cells
G6PD deficiency and thalessemias = Hb precipitation
Bite cells, RBC inclusions (Heinz bodies), inc indirect bili, inc LDH, dec haptoglobin, jaundice, back pain, DARK urine
G6PD deficiency
- activity level may be NORMAL during an attack
Inc marrow cellularity + megakaryocyte hyperplasia
Essential thrombocytosis
Shiny tongue, palmar crease, Hb 7.5, WBC 3500, Hx gastric bypass
B12 deficiency d/t bypass/no IF
Everything inc but TIBC dec
Hemochromatosis
Absent CD55
Paroxysmal Nocturnal Hemoglobinuria
- destruction by complement
Hemarthroses, epistaxis, joint swelling, young age, family bleeding disorder
Hemophilia A (VIII) or B (IX)
Easy bruising, knee swelling after fall, skin ecchymoses, bruising at waistband
Hemophilia (A = VIII, B= IX) –> platelet count and Coag
Cause of hemophilia arthropathy
Iron/hemosiderin deposition –> synovitis & fibrosis
Normocytic, normochromic anemia, low retic, Hb 7-10, normal platelet, WBC, bilirubin
Anemia of prematurity - FTT, tachypnea, lethargy, cyanosis, tachycardia
Weak, abd pain, inc urine prophobilinogen
Acute intermittent porphyria
Purpura, large joint arthritis, N/V, melena, normal PT, PTT, Comp & IgA N-Inc
Anaphylactoid purpura
Bruising, bloody stool, IVH, baby born at home, inc PT
Hemorrhagic disease of newborn = vit K def (II, VII, IX, X)
Venous Hct >65 risk
NEC –> partial exchange transfusion
Washing cells/removing WBCs
Prevent febrile transfusion
HPT w/ acute monoarthritis, chondrocalcinosis
Pseudogout - CPPD = rhomboid crystal, +
Helmet cells
Trauma - DIC, HUS, TTP
Drugs that cause folic acid deficiency
TMP-SMX, MTX, Phenytoin, phenobarbital
Platelet dysfunctions
vWD, renal failure (uremic coagulopathy)
Inc PT (INR)»_space; aPTT
Vit K deficiency
CF + prolonged PT deficiency
Vit K –> low factor VII
Lupus anti-coagulant prolongs?
PTT
Drop of >50% platelets on heparin
HIT –> stop all heparin & use direct thrombin inhibitor + serotonin release assay
Absent Ib-IX-V, mild thrombocytopenia but profuse bleeding, giant platelets
Bernard-soulier
Anemia, thrombocytopenia, HS megaly, BONE PAIN/erosions, PAS+ inclusions
Gaucher’s
Blood transfusions before 1986 and 1992 screened for what?
HBV and HCV, respectively
Paresthesias & hypoCa, hypoMag after receiving several PRBC units
Citrate anti-coagulant in whole blood chelates serum Ca
Pancytopenia, young A.A female, joint pain, rash, 1+ proteinuria, FPGN
SLE –> peripheral immune destruction of all 3 cell lines
Isolated thrombocytopenia, no infections
Immune thrombocytopenia (ITP) —> steroids then splenectomy or rituximab
BM shows inc MEGAkaryocytes
URI weeks ago –> sudden petechiae, epistaxis, thrombocytopenia >30,000
ITP –> Observation; steroids if <30,000
Hemolytic anemia, thrombocytopenia, renal failure, HA, fragmented RBCs
TTP-HUS (ADAMTS-13) —> plasma exchange
Painless blisters on dorsal hand (sun-exposed area),fragility, facial hyperpigment, hypertrichosis, inc uroporphyrins, HCV, estrogen use or iron overload
Porphyria cutanea tarda –> phlebotomy, hydroxychloroquine or IF-a
Deficiency in urobilinogen decarboxylase –> urine porphyrin inc
Porphyria cutanea tarda
1 inherited disorder causing hypercoagulability
Factor V Leiden
HCV, palpable purpura, hematuria, proteinuria, low complement
Mixed cryoglobulinemia = Hep C
Palpable purpura on butt of kid
Henoch-Schonlein
Purpura, normal platelet count, arthralgia, abd pain/bleeding, scrotal swelling
Henoch-Schonlein purpura –> intussusception = IVFs, NSAIDs or steroids –> SURGERY
Hematuria, abd pain, purple non-blanching rash on lower legs, normal platelets
Vasculitis/HSP
Senile purpura
Perivascular CT atrophy
Therapy for iron overload in B-thal or sickle cell
Deferoximine
Ruby papules on lips, high Hct, recurrent nosebleeds, digital clubbing
Osler-Weber-Rendu –> AVMs (lungs –> shunt –> polycythemia, hemoptysis)
Delayed clamping of umbilical cord –> ?
Polycythemia –> Resp distress
Resp distress, poor feeding, neuro sx
Polycythemia
Pruritus after warm shower, ulcers, facial plethora, Low EPO
PCV
JAK2 mutation
PCV
PNA sx but lymphocytosis, smudge cells, how to confirm Dx?
CLL –> flow cytometry
Most common leukemia in Western countries
CLL
Elderly, lymphocytosis, smudge cells (breakdown/inc fragility)
CLL
Reed-Sternberg cells
Hodgkins lymphoma
Leukocytosis with neutrophilia, marked splenomegaly, low leukocyte alkaline phosphatase
CML
Pt got chemo & rad for Hodgkin lymphoma –> CXR density dx?
Secondary malignancy - also breast, bone, thyroid, GI cancers
Numerous infections, weight loss, HSM, LAD, petechiae
ALL = higher in boys, Down
Pale, petechiae, fever, fatigue, rubbery non-tender LNs, anemia, widened mediastinum
ALL –> BM Bx
LAD, splenomegaly, 80% blasts, Inc prolymphocytes, (-) PAS (+) Tdt
ALL
Starry sky appearance
Burkitt lymphoma
Tx for acute limb ischemia
Heparin –> surgery –> intra-arterial fibrinolysis
Anti-cardiolipin Abs, recurrent thromboses
Antiphospholipid Ab syndrome
Poor prognostic indicator in CLL
Thrombocytopenia
Cause of death in CLL pts
Infection
Temp 101.3, neutrophils 850
Neutropenic fever –> PA –> Zosyn
Dry tap, TRAP stain, CD11c
Hairy cell leukemia (B) –> Cladribine (purine)
NHL Tx
CHOP
CLL tx
Chlorambucil & Prednisone
BCR/ABL
CML
- Hx of radiation exposure
9;22 activity
TK in CML, tx = Imatinib
Hypocellular + Fibrotic marrow
Myelofibrotic disorders
Retinoic Acid receptor
AML (promyelocytic)
Excess bone resorption in cancer
PTHrP - Breast CA
Excess Ca reabsorption in cancer
Lymphoma (Vit D)
Anterior mediastinal masses
Thymoma - Teratoma - Thyroid - Terrible lymphoma
Mediatinal mass w/ high b-HCG, normal AFP
Seminoma
Mediastinal mass w/ elevated b-HCG, elevated AFP
Mixed germ cell/Non-seminomatous - yolk, chorio
Back pain, anemia, renal dysfxn, elevated ESR, constipation
MM –> hyperCa
Plasmacytosis, monoclonal proteins, lytic lesions
MM (IgG or IgA)
High serum protein, normal albumin (gamma gap) disorders
MM, amyloidosis, waldenstroms, MGUS
Gamma gap, anemia, pancytopenia, hyperCa, renal disease
MM
MM SPEP
> 3g M protein, >10% plasma cells in BM
MGUS SPEP
need sketelal x-rays to monitor for –> MM
IgM spike, anemia, bruises, night sweats, dizzy, blurry vision, numbness
Waldenstroms
Metabolic/electrolyte abnormalities w/ tumor lysis syndrome
HypoCa, HyperPhos, Uric acid, K
Frequency of brain mets by cancer type
Lung > Breast > Melanoma > Colon
Solitary brain mets
Breast, Colon, RCC
Multiple brain mets
Lung, Melanoma
Pappenhiemer bodies
Sideroblastic anemia - phagocytes ingest excessive iron
LOW Hb and haptoglobin
Inc LDH and bilirubin
Portal vein thrombosis
PNH
- no GP1 –> cant bind CD55 or 59 –> destruction by complement
- Dx by flow cytometry
Signs and sx of polycythemia in newborn
RESPIRATORY DISTRESS
Poor feeding, hypoglycemia, hypoCa
Tx = hydration or partial exchange transfusion