Heme Flashcards

Question

What is extravascular hemolysis?

Answer 1

RBCs are predominantly destroyed by phagocytes in the reticuloendothelial system (eg LN, spleen) Can be due to antibody-mediated RBC destruction (most cases of AIHA) or intrinsic RBC enzyme (G6PD deficiency) or membrane (eg hereditary spherocytosis) defects Normal to slightly low haptoglobin, slightly elevated LDH, elevated indirect bilirubin AIHA - negative fhx, positive coombs test Hereditary spherocytosis - positive fhx, negative coombs test

Answer 2

Oral direct 10a inhibitors (rivaroxaban, apixaban) Rapid onset of action, no requirement for lab (INR) monitoring, no requirement for overlap therapy w/ heparin; do not increase risk of bleeding complications Contraindications: impaired renal function or DVT/PE secondary to malignancy

Answer 3

Typically reserved for hemodynamically unstable (hypotension, tachycardia) pts w/ PE. Less commonly used for massive proximal DVT assoc. w/ significant sx swelling and/or limb ischemia.

Answer 4

Most common cause of vit. B12 deficiency (macrocytic anemia, glossitis, peripheral neuropathy) Due to: anti-intrinsic factor antibodies, or development of chronic atrophic gastritis w/ decreased production of IF by gastric parietal cells Assoc. w/ at least double the risk of gastri cancer compared to general population.

Answer 5

15-35, >60yo Fever, fatigue, painless cervical and supraclavicular LAD, pruritis (a paraneoplastic process classically assoc. w/ HL and likely due to cytokine and interleukin release) Mediastinal LAD can compress adjacent structures and lead to cough, SOB, and retrosternal chest pain Alcohol may exacerbate pain Usually arises in a single LN and disseminates to contiguous nodes via lymphatic and thoracic ducts Labs: normocytic anemia, eosinophila (tumor cells secrete cytokines that generate eosinophils), lymphopenia, elevated LDH (assoc. w/ high tumor burden) Dx: exicisonal lymph node biopsy (reed sternberg cells are diagnostic)

Answer 6

Fever, fatigue, peripheral LAD, sore throat; resolves in a month tender LAD splenomegaly

Answer 7

Pts present w/ joint pain and swelling following little or no trauma due to spontaneous bleeding into a joint (hemarthrosis). Recurrent hemarthrosis can result in long-term complications such as hemophilic arthropathy, joint damage caused by intra-articular bleeding. Hemosiderin deposition w/in jt triggers synovial inflammation, which leads to fibrosis and destruction of cartilage and bone. Visible on XR, MRI allows for earlier detection and characterization of degree of jt damage Early prophylaxis w/ factor concentrates can significantly reduce risk of developing arthropathy BM evaluation is contraindicated in severe hemophilia due to bleeding risk.

Answer 8

Presentation: obesity, snoring, daytime somnolence Short periods of hypopnea and apnea reduce blood O2 levels. Kidneys respond to hypoxemia by increasing erythropoietin. EPO stimulates BM to differentiate mor RBCs. Therefore, it is quite common for pts w/ OSA to have elevated hematocrite (polycthemia).

Answer 9

Due to reduced plasma volume.

Answer 10

Erythrocytosis. Due to increased RBC mass and can be primary (polycthemia vera) or secondary (due to chornic hypoxia or EPO producing tumors).

Answer 11

Increases carboxyhemoglobin levels. This shifts the O2 dissociation curve to the left, resulting in tissue hypoxia. Kidneys respond by increasing EPO, which may cause erythrocytosis. Pts w/ CO poisoning typically present w/ dizziness, headaches, nausea, and confusion. Severe: seizures Pulse ox is unreliable and may appear normal b/c it cannot differentiate carboxyhemoglobin from oxyhemoglobin. Tx: 100% O2 via nonrebreather facemask Hyperbaric O2 used in severe cases that are unresponse to facemask administered O2

Answer 12

Abdominal pain + neuropsych issues (psychosis, neuropathy) Occurs early in porphyrin synthesis Caused by partial deficiency of porphobilinogen deaminase, an enzyme involved in heme synthesis Patchy sensorimotor neuropathy and autonomic dsyfunction

Answer 13

A form of treatment for a diseae when a standard treatment fails.

Answer 14

Treatment given in addition to standard therapy. | -radiation therapy given at the same time as radical prostatectomy

Answer 15

An initial dose of treatment to rapidly kill tumor cells and send the patient into remission (<5% tumor burden) -induction therapy for acute leukemia

Answer 16

Typically given after induction therapy w/ multidrug regimens to further reduce tumor burden. -multidrug therapy after induction therapy for acute leukemia

Answer 17

Usually given after induction and consolidation therapies (or initial standard therapy) to kill any residual tumor cells and keep pt in remission. -daily antiandrogen therapy for prostate cancer

Answer 18

Treatment given before the standard of therapy for a particular disease. -radiation therapy given before radical prostatectomy was done

Answer 19

Most common childhood cancer Bone pain (caused by leukemic infiltration of BM) LAD and hepatosplenomegaly due to extramedullary leukemic spread. Labs: low platelets, anemia Dx: bone marrow biopsy w/ 25% lymphoblasts Tx: multidrug chemo

Answer 20

Fever, splenomegaly, bone pain No assoc. w/ lymphadenopathy Most common cause of pediatric stroke, dx confirmed by hemoglobin electrophoresis.

Answer 21

Pts w/ antiphospholipid antibodies (lupus anticoagulant) generally have prolonged PTT b/c antiphospholipid antibodies bind the phopholipids used in most testing assays and prevent them from inducing coagulation.

Answer 22

Pure red cell aplasia w/ reticulocytopenia and normal platelet and WBC counts Caused by congenital (inherited or sporadic) defect of erythroid progenitor cells, which leads to increased apoptosis of RBCs Macrocytic anemia, craniofacial anomalies, triphalangeal thumbs, short stature, cleft palate, webbed neck Tx: corticosteroids or transfusion therapy in refractory cases

Answer 23

Autosomal recessive Pancytopenic bone marrow failure Most common cuase of congenital bone marrow failure due to defective DNA repair In addition to pancytopenia, characteristic features include short stature, hypoplastic thumb, polydactyly or a flat thenar eminence, and abnormal skin pigmentation Dx: made by demonstrating chromosomal breakage following DNA exposure to interstrand crosslinking agents Tx: definitive - hematopoietic stem cell transplantation

Answer 24

Due to quantitaive or qualitative vonWF deficiency Presentation: mucocutaneous bleeding (epistaxis, gingival bleeding, postpartum hemorrhage) Factor 8 is more rapidly degraded when unbound leading to prolong aPTT However pts can have normal aPTT: mild to moderate deficiency w/ adequate factor 8, vWF and F8 are acute phase reactants (stress + inflammation can acutely raise levels), vWF synthesis is increased by estrogen and TH (pregnancy, ocps, TH replacement can raise vWF and normalize aPTT) Plt count and PT are normal Impaired plt function = prolonged bleedint time Dx: vWF antigen level, vWF activity (ristocetin cofactor activity), F8 levels Tx: desmopressin potentiates release of vWF from endothelial cells and can be used in mgmt of acute bleeding or for prophylaxis

Answer 25

A B cell neoplasm assoc. w/ elevated monoclonal IgM High levels of IgM may cause hyperviscosity syndrome (visual changes, HA, vertigo, dizziness, ataxia), peripheral neuropathy, cryoglobulinemia, and/or renal insufficiency Dx: serum protein electrophoresis and bone marrow biopsy

Answer 26

Significant leukocytosis (often >100,000) LAD B symptoms

Answer 27

Hepatomegaly, LAD, + B sxs are UNCOMMON

Answer 28

Alcohol abuse is the most common cuase of nutritional folic acid deficiency in the US Deficiency occurs quickly (5-6w) due to naturally low stores. Normal methylmalonic acid levels. No neurologic manifestations.

Answer 29

Common after a total of partial gastrectomy. Necessary cofactor in purine synthesis, and its deficiency causes defective DNA synthesis, which results in ineffective erythropoiesis, presenting as megaloblastic anemia Glossitis, pale palmar crease Takes years (not months) to develop. HIGH methylmalonic acid levels.

Answer 30

Autosomal dominant Hemolytic anemia, jaundice, splenomegaly High MCHC (due to membrane loss and RBC dehydration) Negative coombs test, increased reticulocytes in response to RBC destruction Spherocytes on peripheral sphere Due to their shape, these abnormal RBCs get trapped and destroyed in the spleen Tx: supportive care, transfusions; splenectomy for severe hemolysis

Answer 31

X linked recessive Jaundice, splenomegaly Elevated reticulocytes, negative coombs test, LOW MCHC Heinz bodies leading to bite cells Tx: avoidance of hemolytic triggers rather than splenectomy

Answer 32

Typically presents w/ microcytic anemia and normal RDW. | An elevated reticulocyte count reflects a compensatory bone marrow response to the anemia.

Answer 33

Most commonly inherited hypercoaguable disorder in the white population, leading to increased risk of thrombosis. Testing should be considered for an unprovoked 1st time thrombus in young (<45) pts or those w/ an unsual site of thrombus.

Answer 34

Most common porphyria Due to abnormalities in later steps of heme synthesis Photosensitivity due to accumulation of porphyrinogens that react w/ O2 on excitation by UV light Due to deficiency of UROporphyrinogen decarboxylase Blisters that heal w/ scarring, hyperpigmentation, triggered by estrogens, common in pts w/ hep C Dx: elevated plasma or urinary porphyrin levels Tx: phlebotomy or hydroxychloroquine may provide relief, as can tx for hep C

Answer 35

Classic thrombotic complication: Budd-chiari syndrome, a disorder marked by hepatic venous outflow obstruction (usually due to hepatic vein thrombosis) -progressive discomfort, ascites, hepatosplenomegaly

Answer 36

Pts w/ autoimmune hemolytic anemia have tendency for venous thromboembolism, but those with PNH are at particular risk esp. w/in intraaabdominal or cerebral veins. Should be suspected in pts who have a combination of hemolytic anemia, cytopenias, and hypercoaguable sate (intraabdominal or cerebral venous thrombosis) Flow cytometry tests are used to confirm the diagnosis by assessing for absence of the CD55 and CD59 proteins on the surface of RBCs.

Answer 37

A disorder of iron utilization that most commonly occurs in setting of chronic inflammation. Characterized by normocytic anemia Low FE, low TIBC, low iron saturation Normal/elevated serum ferritin (keeping Fe stored away) Treating underlyling inflammatory disorder improves anemia

Answer 38

Most commonly occurs 5-10d after an acute, often bloody, diarrheal illness caused by shiga-toxin producing strain of e. coli Presentation: edema, bruising, bloody diarrhea, jaundice Labs: anemia, thrombocytopenia, elevated cr Smear: schistocytes Treatment of prodromal diarrhea w/ antibiotics or antimotility agents (eg loperamide) can increase risk of developing HUS and is not recommended.

Answer 39

May initially present w/ symptoms related to metastatic disease. Spread to axial skeleton is common and manifestation typically include slowly progressive lower back pain or functional impairment (eg motor weakness, incontinence) Prostate cancer usuallly causes osteoblastic bone lesions w/ normal or low ca, elevated ALP, and XR evidence of focal, sclerotic lesions.

Answer 40

Occurs as a response to severe infection, which mobilize mature and immature leukocytes, and is marked by leukocyte counts >50,000. Compared to chronic myeloid leukemia, LR is characterized by the presence of a high alkaline phosphatase score, a greater proportion of late neurtorphil precursors (metamyelocytes, bands) rather than early (promyelocytes, myelocytes), and a lack of absolute basophilia.

Answer 41

Often causes an anterior mediastinal mass Growth can compress adjacent stuctures. No eosinophilia, pain does not worsen w/ alcohol

Answer 42

Can cause anterior mediastinal mass and can compress adjacent structures (leading to sxs) However, most cases arise in 20-40yo not 65yo Eosinophilia is not common, pain does not worsen w/ alcohol

Answer 43

Characterized by increased HbA2 and HbF on electrophoresis due to absent beta globin expression. Since HbF predominates first few months of life until HbA is synthesized, beta thalassemia major is generally asymptomatic in newborn period. Around 6-12mo, pts develop fatigue and pallor due to microcytic anemia Splenic hemolysis of RBCs: jaundice, dark urine, splenomegaly If left untreated pt can develop skeletal abnormalities due to extramedullary hematopoiesis Pts are transfusion-dependent and at risk for iron overload. Therfore, chelation therapy is required to improve survival.

Answer 44

The proximal deep leg veins (femoral*, iliac, popliteal) are the most common source of symptomatic PE - 90% due to their large caliber and proximitiy to the lungs Other less common sources of emboli include the calf veins, renal veins, pelvic veins, upper extremity veins, and right heart - less likely to embolize and more likely to spontaneously resolve.

Answer 45

Pts w/ a DVT/PE who have a contraindication to or complicatoin from anticoagulation (eg significant active bleeding, recent surgery, acute hemorrhagic stroke) should have an IVC filter placed. Another indiciation for placement includes if anticoagulaton fails (eg the pt develops a new or recurrent proximal DVT while on therpeutic anticoagulation) In a pt w/ life-threatening GI bleed (w/ a therapeutic INR and a normat plt countt), therapeutic anticoag (ie warfarin, heparins, rivaroxaban) is contraindicated

Answer 46

Anemia can be due to decreased RBC production, increased RBC destruction, and frank blood loss. The anemia of lymphoproliferative disorders is due to bone marrow infiltration w/ cancerous cells.

Answer 47

RBCs w/ irregularly sized and spaced projections that are most commonly seen in liver disease.

Heme Flashcards

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