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54 Cards in this Set

  • Front
  • Back
is shigella associated with neutropenia?
no
6 bacterial infections associated with neutropenia
brucellosis
gram negative sepsis
paratyphoid fever
typhoid fever
tularemia
TB
4 mechanisms of neutropenia in bacterial infection
complement mediated margination of neutrophils along endothelial surfaces
increased utilization of neutrophils
marrow failure
generation of IL-8, TNF alpha, IL-1beta (leads to margination)
what test would you do to evaluate suspicion of immune mediated neutropenia?
anti-neutrophil antibody
when does immune neutropenia of infancy resolve?
spontaneously after median of 17 months
what is kostmann's syndrome?
severe congenital neutropenia
what does bone marrow show in kostmann's syndrome?
maturation arrest at promyelocyte-myelocyte stage
what mutations have been identified in kostmann's syndrome?
neutrophil elastase (60-80%)
WASP gene mutation (rare)
GFI-1 (rare)
what is the therapy for Kostmann's syndrome?
G-CSF (95% respond)
what oral physical findings differenetiate kostmann's from immune neutropenia
gingivitis
mouth ulcers
what is the genetic mutation in cyclic neutropenia?
mutation in neutrophil elastase gene
what are the bone marrow findings in cyclic neutropenia
hypoplasia or myeloid maturation arrest
increased number of eosinophils
what comprises shwachman-Diamond Syndrome
pancreatic insufficiency
neutropenia
metaphyseal chondroplasia
chemotaxis defect
what is the genetic mutation in cyclic neutropenia?
mutation in neutrophil elastase gene
what are the bone marrow findings in cyclic neutropenia
what cells can be increased
hypoplasia or myeloid maturation arrest
increased number of eosinophils
what comprises shwachman-Diamond Syndrome
pancreatic insufficiency
neutropenia
metaphyseal chondroplasia
chemotaxis defect
what comprises Cartilage Hair Hypoplasia
AR
short limb
dwarfism
fine hair
moderate neutropenia
dyskeratosis congenita
x linked
nail dystrophy
leukoplakia
reticulated hyperpigmentation
marrow hypoplasia
neutropenia
glycogen storage disease - type 1B: gene defect
impaired intracellular transport of glucose-6-phosphate by G6P translocase from cytosol to ER
what BMP abnormality is seen with glycogen storage disease type 1B
hypoglycemia
job's syndrome aka..
hyperimmunoglobulin E syndrome
pathophysiology of job's syndrome
defective IL12/ interferon gamma pathway leading to defective neutrophil chemotaxis and elevated IgE levels
clinical manifestations of jobs syndrome
eczema
skin infection
sinus infection
pulmonary infection
candidal infection
bone fractures
teeth abnormalities (retention of primary teeth)
hyperextensible joints
delayed cord separation is characteristic of:
leukocyte adhesion deficiency
what cell lines are affected in chediak higashi, what is the abnormality?
defect: giant cytoplasmic inclusion
WBCs:neutrophils, monocytes, lymphocytes
platelets
melanosomes
treatment for chediak-higashi
prophylactic bactrim
parental antibiotics for acute infections
vitamin C
BMT at or before accelerated phase
what does vitamin C do in patients with Chediak-higashi
improve neutrophil motility
what is a pelger huet anomaly? where is it seen
bilobed nuclei in PMD
specific granule deficiency
what infections are individuals with chronic granulomatous disease at risk for developing?
staph aureus
burkholderia
serratia
nocardia
aspergillus
candida
what happens in the NBT test?
originally stain yellow, when neutrophils are activated blue granules appear
what GI/GU findings are seen in CGD
gastric outlet obstruction
urinary tract obstruction
colitis
management of CGD
prophylactic bactrim
itraconzole
interferon - gamma
granulocyte transfusions
gene therapy
BMT
what leukocyte disorder can occur as an acquired defect in M3 AML due to the 15/17 translocation?
myeloperoxidase deficiency
what leukocyte deficiency is associated with diabetes mellitus?
myeloperoxidase deficiency
what is the mechanism of neutropenia from phenytoin and phenobarbitol?
metabolite mediated damage to the neutrophil
what is the mechanism of neutropenia from phenothiazine?
direct toxicity to neutrophils
what is the mechanism of neutropenia from penicillin
immunologic
anti-neutrophil antibody positive
how frequently does a nadir occur in cyclic neutropenia?
21 +/- 4 days
in what disorder is there a reduced conversion of dihydrorhodamine to rhodamine
x linked chronic granulomatous disease
do patients with leukocyte adhesion deficicency have neutropenia or neutrocytosis?
neutrocytosis and leukocytosis
what is the most common presenting feature of neutropenia?
oral mucosal ulcerations and gingivitis
what is the defective gene/protein in chediak higashi?
what chromosome?
LYST found on chromosome 1q13
what is the mutation C/EBP associated with?
specific granule deficiency (sgd)
deficient or reduced expression of CD11/ CD18 family of receptors on the neutrophil surface established the diagnosis of which neutrophil disorder?
leukocyte adhesion deficiency
where does maturational arrest occur in Kostmann's syndrome?
promyelocyte
what cytogenetic abnormality is seen in patients with Kostmann's who progress to MDS or AML?
monosomy 7
what is reticular dysgenesis? what level immunoglobulins seen?
failure of stem cells to form both myeloid and lymphoid precursors
agammaglobulinemia
what ethnic group is cartilage hair hypoplasia seen in?
Amish
Name two leukoyte disorders where dwarfism is a feature.
Schwachman-Diamond
Cartilage Hair Hypoplasia
what is the defect in dyskeratosis congenita?
defect in DNA repair
what is the accelerated phase in Chediak higashi
massive proliferation of T lymphocytes in the liver, spleen and bone marrow resulting in worsening cytopenias
what inborn errors of metabolism are associated with neutropenia?
glycogen storage disease type 1B
methylmalonic acidemia
isovaleric acidemia
prioponic acidemia
why are WBC and neutrophil counts high in leukocyte adhesion disorder?
receptors (CD11/CD18) on neutrophils that cause adhesion to endothelium of sites of inflammation are deficient or missing. therefore neutrophils cannot exit the circulation and remain in the circulation in increased numbers
name catalase positive organisms
staph aureus
pseudamonous cepacia
serratia marcescens
candida species
aspergillus fumigatus