Heme 2 Flashcards
what level constitutes neutropenia?
decreased neutrophil <1500
With a neutrophil count <100 what increases sharply
infectious diseases
when the neutrophil count is <500 there is impaired what?
impaired control fo endogenous flora
when neutrophil count is <200 what is absent?
inflammatory process is absent
pathophys behind neutropenia
Decreased production (Marrow) Increased sequestration (Spleen) Peripheral destruction/pooling
what drugs caues neutropenia via decreased production
sulfonamides (even bactrim) methotrexate PCN cephalosporins cimetidine phenytoin retrovirals
benign condition where someone have drops in WBC periodically
cyclic neutropenia
what ethnicity tend to has a lower WBC count?
african americans
what nutrient deficiencies can cause neutropenia
folate or Vit B12 deficiency
what infections can lead to neutropenia
TB measles mono viral hepatitis AIDS sepesis
what causes peripheral destruction of neutrophils
anti-neutrophil antibodies
autoimmune disorders (RA, HIV, SLE)
Felty’s syndrome
Wegner’s granulomatosis
what is felty’s syndrome
immune neutropenia + sero (+) nodular RA+splenomegaly)
chronic inflammation + granuloma formation in the nasal pharynx, lung and kidneys
Wegner’s granulomatosis
what cause neutropenia via peripheral pooling
severe bacterial infections
cardiopulmonary bypass
hemodialysis
sequestration (in spleen or liver)
most common infections that occur due to neutropenia
pneumonia
cellulitis
septicemia
clinical presentation of neutropenia
infection
stomatitis
fever
why is a peripheral smear important
confirms what your instrument read
when is a bone marrow biopsy needed w/ neutropenia
really low count and no known reason
tx for felty’s syndrome w/ repetitive infections
splenectomy
what can you do to tx immune mediated conditions causing neutropenia?
immune mediated therapy
A rise in cell count above the normal limits of any leukocyte (myeloid or lymphoid lines).
leukocytosis
what is neutrophilia
absolute count >10,000
what are primary causes of neutrophilia
congenital disorders
myeloproliferative disorders
leukemia
what are secondary causes of neutrophilia
infection (acute/ chronic) acute stress drugs steroids inflammation marrow hyperstimulation splenectomy smoking (mild)
how do you screen for chronic leukemia (chronic myelogenous leukemia)
BCR/ABL
what is BCR/ABL?
genetic mutation
what causes lymphocytosis
reactive increase to viral or fungal infection
allergic rxn
benign autonimmune process
lymphoplasmacytic disorders (luekemia CLL, ALL)
Decrease in platelet count below low normal (<150,000/μL).
thrombocytopenia
3 main mechanisms of thrombocytopenia
Decreased marrow production Increased sequestration (in spleen) Accelerated peripheral destruction
what caused decreased marrow production leading to thrombocytopenia
suppression of megakaryocytopoiesis (marrow damage) from cytotoxic chemo, ETOH
destruction of normal stem cells
intrinsic defects of the stem cells
metabolic abnormalities affecting megakaryocyte maturation
what is a very common cause of low platelets
alcohol abuse
what conditions can cause splenomegaly
Advanced liver disease
Myeloproliferative disorders
Malignancies of the spleen
how many platelets are held in the spleen at once
30%
most common cause of thrombocytopenia (immune mediated)
ITP
what is HIT?
heparin immune mediated thrombocytopenia
usually the 2nd time they get heparin
what are non-immune mediated causes of thrombocytopenia
TTP/HUS DIC HELLP anti-phospholipid syndrome pre-eclampsia
what is HELLP
hemolytic anemia, elevated liver function tests, and low platelet count
how do people w/ thrombocytopenia present
mucosal or dermatologic bleeding epistaxis gingival bleeding GI purpura/ petechiae
labs for thrombocytopenia
CBC w/diff, LFTs, ANA, RF, HIV, TSH
PT/PTT (protime & partial thromboplastin time)
Blood smear: with particular attention to platelet morphology, size, clumping
B12 & Folate levels
Consider Bone Marrow in pts > 60
is an autoimmune disorder which IgG antibodies are formed that bind to platelets which lead to destruction by the spleen
ITP
presentation with ITP
Overall, patients are well and afebrile.
Major complaint is mucosal or skin bleeding
More common in children than adults
what does ITP commonly follow in children
a viral illness
commonly self limited in children
where does ITP tend to occur?
weight dependent areas
look on ankles
does ITP blanch?
No
why are platelets slightly enlarged w/ normal morphology w/ ITP
newer platelets so they are larger
what is evan’s syndrome
Blood smear show anemia, reticulocytosis and spherocytes
first line of tx w/ ITP
prednisone
what are other txs for ITP?
IVIG, Winrho (Rho-Gam) Rituxan
definitive tx for ITP
splenectomy
what is a treatment for ITP that is new. It is a growth factor and helps encourage bone marrow to make platelets. ONly works while you give it.
weekly injection
Nplate (Romipostim)
what is TTP?
Thrombotic Thrombocytopenic Purpura
Non-Immune cause of thrombocytopenia accompanied by microangiopathic hemolytic anemia and increased serum LDH
what is the pentad w/ TTP
- Thrombocytopenia (bruising or purpura)
- Microangiopathic hemolytic anemia (anemia, jaundice and a blood film featuring evidence of mechanical fragmentation of red blood cells)
- Neurologic symptoms (fluctuating), such as hallucinations, bizarre behavior, altered mental status, stroke, or headaches
- Kidney failure
- Fever
lab findings w/ TTP
anemia
reticulocytosis
smear shows microangiopathic picture
Tx for TPP
plasmapharesis
prednisone and ASA as adjunct
what is the definitive test for TTP?
ADAMTS13 (protease) is low
takes 2 weeks for result
More common cause of non-immune platelet destruction
DIC
what causes DIC
sepsis severe tissue injury (excessive surgery) obstetric complications cancer severe hepatic illness
labs for HUS
Thrombocytopenia (usually less severe) RBC fragmentation (a must have) Elevated LDH/Negative Coomb’s Coagulation tests normal Some increase in fibrin degradation Renal biopsy can be performed for Dx Necrosis and thrombi
what should raise a flag for DIC?
Spontaneous bleeding or oozing following needle stick should raise a flag
tx for DIC
fibrinogen with cryopercipitate
fresh frozen plasma
platelet transfusion
if you give RBCs what else do you need to do?
give FFP and platelets too
WBCs may increase to 10-25,000
RBCs usually normal
thrombocytosis
DDx for thrombocytosis
Polycythemia vera
reactive thrombocytosis
CML
complications w/ thrombocytosis
thrombosis and or embolus
stroke, MI, DVT, PE
hemorrhage may be severe
tx for thrombocytosis
ASA
anticoagulation (plavix)
what do you use to treat essential thrombocytothemia (thrombocytosis)?
hydroxyurea
anagrelide
interferon
Excess production of all cell lines, esp. RBC
polycythemia vera (PCV)
Primarily excess platelet production
Essential Thrombocythemia (thrombocytosis)
Fibrosis of marrow with decreased cell production
myelofibrosis
1st presenting sign of polycythemia vera
splenomegaly
Pruritis with warm water exposure
if left unchecked what can happen w/ polycythemia vera?
Neurological symptoms
Systolic BP increased ? viscosity
Arterial/Venous thrombosis can be a clinical indicator
Paradoxical presentation
test for polycythemia vera?
Jak 2 positive
tx for polycythemia vera
phelbotomy
low diet in Fe can be helpful
myelosuppressive therapy
should you put someone with polycythemia vera on iron even if levels are low?
NO!
what is a tx option for polycythemia treatment in older patients
hydroxyurea
what other drugs may be needed w/ polycythemia vera
allopurinol
anithistamines
Uncommon Myelo Proliferative Disorder of unknown cause with marked proliferation of megakaryocytes in the bone marrow leading to high platelet count
Essential Thrombocytothemia
most common presentation of essential thrombocytothemia (thrombocytosis)
Thrombosis (random blood clots) commonly occur in mesenteric, hepatic, or portal vein
what will bone marrow look like for essential thrombocytothemia (thrombocytosis)
increased numbers of megakaryoctyes w/ normal morphology
Tx for thrombocytothemia (thrombocytosis)
hydroxyurea (want platelets <500,000)
Anagrelide (poorly tolerated)
ADRs w/ anagrelide (tx for essential thrombocytothemia (thrombocytosis)
HA, mild anemia, peripheral edema
CHF w/ high doses
characterized by fibrosis of the bone marrow, splenomegaly and leukoerythroblastic peripheral blood picture with poikylocytosis
Fibrosis of the marrow believed to be a response of platelet-derived growth factor and other cytokines
Myelofibrosis
where does hematopoiesis take place with myelofibrosis
the liver, spleen and lymph nodes (as compensation)
how does someon with myelofibrosis present
Over 50
fatigue (anemia), abdominal fullness, splenomegaly, hepatomegaly (50%)
Whey does portal HTN occur later in the course w/ myelofibrosis?
secondary to hepatic hematopoiesis
What will the blood smear look like w/ myelofiborsis (triad)
poikylocytosis
luekoerythroblastic blood
giant degranulated platelets
Tx for myelofibrosis
transufions, splenectomy
alpha interferson
what helps to reduce spleen size w/ myelofibrosis
jakafi
Idiopathic malignant proliferation of lymphoid stem cells in bone marrow that invade lymph nodes, spleen, liver, and other organs
Classed as B or T cell
Acute Lymphoblastic Leukemia (ALL)
who is mostly affected by ALL
young children (most common childhood malignancy)
Usually in adults over 50 years
Male:Female (2:1)
proliferation of mature-appearing lymphocytes in peripheral blood that invade bone marrow, spleen, lymph nodes
Usually B-lymphocytes (>90%)
Chronic lymphocytic leukemia (CLL)
what is the most common form of chronic luekemia?
CLL
Presentation of CLL
Complaints of weakness, fatigue, Dyspnea On Exertion, infections
PE: lymphadenopathy, hepatosplenomegaly
What chromosome abnormality may a patient with CLL Have?
trisomy 12
What is the staging system used for CLL?
Binet
CLL stage lymphocytosis w/10 yrs. No tx if asymptomatic
Binet Stage A
stage of CLL with anemia/thrombocytopenia despite number of lymph node groups; mean survival 2 yrs; Chlorambucil w/prednisone; splenic irradiation
Binet Stage C
CLL stage with 3 lymph node groups; Mean survival 5 years; Use alkylating agent and radiation to symptomatic nodes
Binet Stage B
Primarily adults >50 yrs (can see in children)
Can be preceded by CML, PV, or other MPD and caused by chemo, chromosomal abn, radiation toxins, drugs or even idiopathic.
Acute Myelogenosu Leukemia (AML)
what is there a proliferation of with AML?
abnormal myeloid cells (precurosrs) that don’t mature
How is AML classified?
FAB (7 subtypes)
Leukemia that presents w/ recurrent infection, bone pain, bruising, bleeding, weakness, bone tenderness.
AML
what is pathognomic for AML on labs
Auer rods
Peak 30-50 yrs, etiology unknown
Proliferation of immature granulocytes
has chronic and acute phases.
Chronic Myelogenous Leukemia (CML)
what phase of CML is where WBC >100,000/mm3 w/ <5% blasts
chronic phase
What phase of CML is where phase >5% blasts and more malignant phase w/WBC increase w/anemia and thrombocytopenia
acute blastic phase
what is present on labs w/ CML
Philadelphia chromosome
Tx for chronic phase of CML
tyrosine kinase inhibitors
Malignant disorder of lymphoreticular system Characterized by “Reed-Sternberg” cells Bimodal age distribution 20-40 year olds > 50 year olds
Hodgkin’s Lymphoma
Complain of painless lump (in neck), fever, drenching night sweats, weight loss, (B symptoms) malaise, generalized pruritis
Hodgkin’s lymphoma
what may cause pain in lymph nodes w/ hodgkin’s lymphoma?
ETOH
What will be elevated on labs w/ hodgkins?
ESR and LDH
what will be decreased on labs w/ hodgkins?
lymphocytes and platelets
stage of hodkin’s with 2 or more lymph node regions on same side of diaphragm
Stage II
Stage with Hogdkin’s with diseeminated involvement of one or more extra-lymphatic organs w/ or w/o node involvement
Stage IV
Stage of Hodgkin’s w/ single lymph node region involvement or single extra-lymphatic site.
Stage I
Stage of Hodgkin’s w/ lymph nodes regions on both sides of diaphragm
Stage III
Tx for stage I-II of Hodgkin’s
2 cycles ABVD chemo + XRT
Tx for stage III, IV of Hodgkin’s
chombo chemo + radiation to sites of bulky dz
Malignancy of the lymphoreticular system
Classified as B, T or U (undifferentiated)
median age is 50
increased incidence w/ HIV
Non-hodgkin’s lymphoma
Non-hodgkin’s presentation
Persistent, painless lymphadenopathy in 2/3 of patients
pruritis (rare)
Tx for Hodgkin’s
Chemo + radiation, depends on stage
Malignant proliferation of plasma cells where of the 5 classes of immunoglobulin produced, one class is produced in excess more often in men and AA
Multiple myeloma
presentation of multiple myeloma
Bone pain (back and fibs), pathological fractures weakness, fatigue, N/V, bleeding, bruising
how is dx of multiple myeloma made?
serum or urine protein electrophoresis then confirmed w/ bone marrow
Tx for multiple myeloma
chemo, radiation, prednisone
