21. Hematologic Alterations Flashcards
presence of blasts cells (immature/nonfunctional WBC) on a blood smear
leukemia
common clinical manifestations of leukemia
- anemia -> fatigue - bleeding -> skin, gums, MM, GI tract, petechiae, ecchymosis - infection -> fever - weight loss/anorexia - bone pain - liver, spleen, and lymph node enlargement - elevated uric acid levels
most common type of macrocytic anemia and is caused by vitamin B12 deficiency
pernicious anemia (PA)
hereditary (AR) factor 9 deficiency; will have prolonged PTT
hemophilia B
5 types of normocytic-normochromic anemias
- aplastic - posthemorrhagic - acquired hemolytic - hereditary hemolytic - anemia of chronic inflammation
erythrocytes that contain iron-laden mitochondria (iron granules) arranged in a circle around the nucleus rather than into Hgb
ringed sideroblasts
aggressive, fast growing leukemia with too many lymphoblasts (immature WBC) found in the blood and BM
acute lymphocytic leukemia (ALL)
cardiac compensatory mechanisms for anemia
- tachycardia - increased stroke volume - capillary dilation
clinical manifestations of SAs
- CV and respiratory manifestations - hemochromatosis (iron overload) - mild-moderate hepatosplenomegaly - bronze-tinted skin may occur
3 phases of CML
- chronic phase (asymptomatic) - accelerated phase (primary sxs develop) - termal blast phase (blast crisis) -> survival of 3-6 months later stages resemble acute leukemia but w/ more prominent and painful splenomegaly
elevated reticulocyte count
hemolytic anemia
explain how DIC causes clots
massive activation of clotting cascade -> widespread microvascular thrombosis and vascular occlusion -> ischemic tissue damage
anemias that result due to ineffective erythrocyte DNA synthesis -> due to vitamin B12 or folate deficiencies
macrocytic (megaloblastic) anemia
old lab test that evaluated B12 absorption by administering radioactive B12 and measuring urine excretion
Schilling test
malignant lymphoma that progresses from one group of lymph nodes to another, including systemic symptoms and presence of B cells called Reed-Sternberg (RS) cells
Hodgkin lymphoma
reduction in the total number of erythrocytes in circulating blood or a decrease in the quality or quantity of hemoglobin
anemia
common causes of anemia
- impaired erythrocyte production - blood loss (acute or chronic) - increased erythrocyte destruction - combo of these 3 factors
ex. of acquired hemolytic anemia
improper blood transfusion
symptoms of B cell lymphomas (HL, NHL, can be seen in LL)
- fever - night sweats - weight loss
Dx of SAs
confirmed by bone marrow bx and presence of sideroblasts
anemias characterized by erythrocytes that are unusually large in size, thickness, and volume
macrocytic (megaloblastic) anemia
anemias characterized by abnormally small erythrocytes that contain abnormally reduced amounts of Hgb
microcytic-hypochromic anemias
Normal RBC level
4.2-6.1 million cells/mcL
reduction in all cellular components of the blood
pancytopenia
most common type of anemia throughout the world; depletion of iron stores and reduced Hgb synthesis
iron deficiency anemia
excessive RBC production
polycythemia
platelet count less than 150,000 platelets/uL
thrombocytopenia
hyperdynamic circulation from increased ECF and SV leads to what cardiac problems
- cardiac murmurs - high-output heart failure
2 causes of IDA
- inadequate dietary intake - chronic blood loss
later stage of CLL where cancer is found in the lymph nodes as well
small lymphocyte lymphoma (SLL) or Non-Hodgkins lymphoma
fast-growing B cell tumor that is more common is Africa
Burkitt lymphoma
leukemia most commonly seen in children
ALL
anemia due to damage to bone marrow erythropoiesis (bone marrow failure)
aplastic anemia
lab findings of HH?
- elevations in serum iron levels and ferritin levels (iron in hepatocytes) - decreased TIBC (transferrin is saturated)
clinical manifestations of IDA
- typical anemia symptoms - nails become brittle and “spoon-shaped” - tongue papillae atrophy (soreness/redness/burning) - gastritis - neuromuscular changes (numbness/tingling) - irritability and headache
although the hallmark of HIT is thrombocytopenia, what else are patients at risk for?
thrombosis due to activation, aggregation, and consumption of platelets
symptoms of TTP (5)
- extreme thrombocytopenia (<20,000) - intravascular hemolytic anemia - ischemic signs and symptoms (most often in CNS) - kidney failure - fever
Normal hematocrit (Hct) level
38-54%
leukocytosis vs leukopenia
- leukocytosis: WBC count is high - leukopenia: WBC count is low
vitamin K is required for synthesis of which clotting factors
2, 7, 9, 10, protein C, and protein S
Endemic and sporadic locations of Burkitt lymphoma
- endemic: mandible - sporadic: pelvic/peritoneum
common AR disorder of iron metabolism and is characterized by increased GI iron absorption and tissue deposition
hereditary hemochromatosis (HH)
common presentation of MM
- hypercalcemia - renal failure -> secondary to hypercalcemia and Bence Jones proteins - anemia - bone lesions - Bence Jones protein in urine (Ig fragments formed by bone lesions)
Tx for sickle cell crisis
- pain control - O2 - rehydrate (dehydration major cause)
multisystem disorder (defect of ADAMS 13) characterized by thrombotic microangiopathy (TMA) -> platelets aggregate and cause occlusion of arterioles and capillaries within microcirculation -> increased platelet consumption and tissue ischemia
thrombotic thrombocytopenic purpura (TTP)
nodal involvement for non-hodgkin lymphoma
localized to single axial group of nodes
stem cell disorder w/ hyperplastic and neoplastic BM alterations that causes uncontrolled proliferation of RBCs (normally due to JAK2 mutation)
polycythemia vera (PV) or primary polycythemia
ex of hereditary hemolytic anemia
sickle cell anemia
Normal hemoglobin (Hgb) level
11.7-14.9 g/dL (lower end in females)
physiologic increase in EPO secretion by kidneys in response to underlying systemic disorder (ex. tissue hypoxia)
secondary polycythemia
explain how DIC causes bleeding
- widespread thrombosis causes consumption of platelets and clotting factors - also causes activation of plasmin and fibrinolysis (try to break up clots) -> lysis of clotting factors and inhibition of platelet aggregation OVERALL: platelets and clotting factors all used up from clots but even if they were available -> clotting system would inhibit further clots from forming to stop potential bleeding
4 common symptoms of anemia (due to tissue hypoxia)
- weakness/fatigue - pallor (skin and mucous membranes) - dyspnea on exertion and increased RR - dizziness and syncope
symptoms of pernicious anemia
- weakness/fatigue - paresthesias in feet and fingers (neuro manifestations from B12 deficiency) - difficulty walking - loss of appetite/weight loss - sore tongue that is smooth and beefy red
immature form of neutrophils; elevated in blood during infection (high production from BM)
bands
common node involvement in Hodgkin lymphoma
multiple peripheral nodes - cervical nodes (usual starting site) - axillary nodes - inguinal nodes
clinical manifestations of DIC
- oozing from puncture sites - GI bleeding - weakness/fatigue - cyanosis/hypoxiemia - hematuria/oliguria/renal failure
aggressive, fast growing leukemia w/ too many myeloblasts (immature WBC that are not lymphoblasts) found in the blood and BM
acute myelogenous leukemia (AML)
most common cause of infectious mononucleosis
epstein-barr virus (EBV)
slow-growing cancer in which too many immature lymphocytes are found mostly in the blood and BM
chronic lymphocytic leukemia (CLL)
Most cases of pernicious anemia result from what? How does this cause anemia
- autoimmune/chronic gastritis -> destruction of IF -> malabsorption of B12 in ileum - also caused by total/partial gastrectomy
progressive clonal expansion of B cells, T cells, or NK cells (mostly B cells)
Non-Hodgkin lymphomas
heterogenous group of inherited or acquired disorders characterized by anemia of varying severity and presence of ringed sideroblasts in bone marrow
sideroblastic anemias (SAs)
anemia caused by sudden blood loss w/ normal iron stores
posthemorrhagic anemia
Normal WBC count
5,000-10,000
clinical manifestations of PV
- unique feature: aquagenic pruritus (painful itching when exposed to heat or water) - red color in face, hands, feet, ears, and mucous membranes - engorgement of retinal and cerebral veins -> cerebral thrombosis likely - HA, delirium, mania, psychotic depression, chorea, visual disturbances
lymphoid progenitors
B cells and T cells
leukemia caused by BCR-ABL fusion gene derived from reciprocal translocation of chromosomes 9 and 22 (Philadelphia chromosome)
CML
rare variant of NHL but more common in children; vast majority is of T-cell origin
lymphoblastic lymphoma (LL)
refers to hemoglobin content
-chromic
causes of DIC (5)
- trauma - sepsis - cancer - products of conception - injury to endothelium
renal compensatory mechanisms for anemia
- RAAS - increased salt and H2O retention - increased extracellular fluid
benign, acute, self-limiting, lymphoproliferative syndrome characterized by acute infection of B cells and usually transmitted through saliva
infectious mononucleosis (IM)
common causes of prolonged PTT
Heparin therapy Hemophilia
essential (diet dependent) vitamin required for RNA and DNA synthesis in maturing erythrocytes
folate (folic acid)
Normal PT range
10-13 seconds
immature RBC released from bone marrow; still have nucleus; should not be too many in blood
reticulocyte
acquired clinical syndrome characterized by widespread activation of coagulation resulting in formation of fibrin clots in microvasculature throughout body
disseminated intravascular coagulation (DIC)
EBV is linked to which lymphoma
Burkett’s lymphoma
Dx for Hodgkin’s lymphoma
- lymph node bx - chest CT/MRI to see extent of nodal involvement
On CBC diff, which cells have the 2 highest values?
- neutrophils (highest) - lymphocytes (2nd)
leukemia caused by transformed precursor B cells in children and mixture of B cell or T cell precursors in adults
ALL
molecule produced by split products of fibrin clot degradation
D-dimer
most common clinical finding in patients w/ CLL
lymphadenopathy (most are asymptomatic)
myeloid progenitors
- basophils - eosinophils - neutrophils - monocytes - erythrocytes - platelets
elevated fibrin degradation products (FDP) and D-dimer are hallmark findings of what?
DIC
2 types of microcytic-hypochromic anemias
- iron-deficiency anemia (IDA) - sideroblastic anemias (SA)
most common cause of thrombocytopenia secondary to increased platelet destruction; usually secondary to infections or other conditions that cause large amounts of antigen in the blood -> immune complex w/ platelets -> destruction
immune thrombocytopenic purpura (ITP)
hereditary factor 8 deficiency; will have prolonged PTT
hemophilia A
T/F: pernicious anemia is a fast acting anemia
False; develops slowly over time (20-30 years)
most common adult leukemia
AML
lab test for folate deficiency anemia
serum folate levels
what type of Hgb content is found in macrocytic anemias
normal Hgb content (normochromic)
PV does what to blood volume, viscosity, and pressure
increases all 3
TIBC is equivalent to what?
transferrin saturation (iron transporter in the blood)
classic symptoms of infectious mononucleosis
- pharyngitis - lymphadenopathy - fever - fatigue (may last 1-2 months after infection) - splenomegaly may occur
manifestations specific to folate deficiency anemia
- cheilosis (scales/fissures in mouth) - stomatitis (inflammation of mouth) - burning mouth syndrome (painful ulcers in buccal mucosa and tongue) - GI problems: dysphagia, flatulence, and watery diarrhea
anemias characterized by erythrocytes that are relatively normal in size and Hgb content but insufficient in number
normocytic-normochromic anemias (NNAs)
anemia caused by immune destruction of erythrocytes
acquired hemolytic anemia
lab findings for IDA
- decreased iron levels - increased total iron binding capacity (TIBC) - transferrin is empty
most treatable version of lymphoma
Hodgkin’s lymphoma
plasma cell (B cell) cancer characterized by slow proliferation of malignant cells w/ tumor cell masses in the BM usually resulting in destruction of the bone
multiple myeloma (MM)
group of bone marrow failure disorders where blood cells in BM don’t mature; precursor to leukemia
myelodysplastic disorder (MDS)
primary source of vitamin K
green leafy vegetables
anemia caused by abnormalities of RBC membrane or cytoplasmic contents; present at birth
hereditary hemolytic anemia
immune mediated, adverse drug reaction caused by IgG antibodies against heparin-platelet factor 4 complex -> platelet activation -> platelet consumption
heparin-induced thrombocytopenia (HIT)
refers to cell size
-cytic
measure of the extrinsic clotting pathway
prothrombin time (PT)
anemia associated with chronic infections, chronic inflammatory diseases, and malignancies
anemia of chronic inflammation
measure of the intrinsic clotting pathway
partial thromboplastin time (PTT)
normal platelet (Plt) count
150,000-300,000
what lab results are seen in PV?
increase RBC and total blood volume
very early and immature form of WBC; sign of leukemia when increased numbers found on blood smear
blast cells
current lab test for PA
methylmalmonic acid and homocysteine levels
slowly progressing disease w/ too many blood cells (not lymphocytes) made in the BM
chronic myelogenous leukemia (CML)
common cause of prolonged PT
Coumadin therapy
Normal PTT range
30-45 seconds (other references say 25-35 seconds)
