Neonatal case conference Flashcards
Letterer-Siwe Disease
More appropriately called Langerhans Cell Histiocytosis (LCH)
Hallmark of LCH is proliferation of and accumulation of bone-marrow derived dendritic cells
No known cause
Peak age of diagnosis is at 2 years of age; however, it can occur at any age
Leukocytosis/Leukemoid Reaction
Leukocytosis defined as elevated number of white cells in the blood
Leukemoid reaction defined as the elevation of normal leukocytes to counts greater than 50,000 cells/μL
The peripheral blood may show small proportions of immature myeloid cells
Leukemoid reaction must be distinguished from chronic myeloid leukemia
Mechanismsof Leukocytosis
INCREASED PRODUCTION IN THE MARROW
Chronic infection or inflammation (growth factor-dependent)Paraneoplastic (e.g., Hodgkin lymphoma; growth factor-dependent)Myeloproliferative disorders (e.g., chronic myeloid leukemia; growth factor-dependent)
INCREASED RELEASE FROM MARROW STORES
Endotoxemia
Infection
Hypoxia
DECREASED MARGINATION
Exercise
Catecholamines
DECREASED EXTRAVASATION INTO TISSUES
Glucocorticoids
Causes Of Neutrophilic leukocytosis
Acute bacterial infections, especially those caused by pyogenic organism; sterile inflammation caused by, for example, tissue necrosis (myocardial infarction, burns)
Causes Of Eosinophilic leukocytosis (eosinophilia)
Allergic disorders such as asthma, hay fever; certain skin diseases (e.g., pemphigus, dermatitis herpetiformis); parasitic infestations; drug reactions; certain malignancies (e.g., Hodgkin and some non- Hodgkin lymphomas); collagen vascular disorders and some vasculitides; atheroembolic disease (transient)
Causes Of Basophilic leukocytosis (basophilia)
Rare, often indicative of a myeloproliferative disease (e.g., chronic myeloid leukemia)
Causes Of Monocytosis
Chronic infections (e.g., tuberculosis), bacterial endocarditis, rickettsiosis, and malaria; collagen vascular diseases (e.g., systemic lupus erythematosus); inflammatory bowel diseases (e.g., ulcerative colitis)
Causes Of Lymphocytosis
Accompanies monocytosis in many disorders associated with chronic immunological stimulation (e.g., tuberculosis, brucellosis); viral infections (e.g., hepatitis A, cytomegalovirus, Epstein-Barr virus); Bordetella pertussis infection
Conditions Associated with Thrombocytosis in the Newborn
acute inflammation (infection, e.g.)
acute blood loss
nutrition-related conditions- iron, itamin E, hyperalimentation with fat emulsions
asplenia
transient myeloproliferative disorder
neuroblastoma
substance abuse in mother during pregnancy
Elevated Platelets Most Consistent with Transient Myeloproliferative Disorder
Transient myeloproliferative disorder originally thought to only occur in patients with trisomy 21
Clinically appears identical to acute myeloid or acute megakaryocytic leukemia
The only distinguishing factor is the spontaneous resolution of all the hematological abnormalities, usually by 12 weeks of age
pustular rash with transient myeloproliferative disorder
The rash is described as papules and vesicles that concentrate on the face and then spread to the trunk
One study described the rash as mimicking erythema toxicum
The skins rashes of true infant leukemia have been described as only nodular
Findings supporting congenital leukemia
Skin lesions consistent-leukemic cell infiltrate into the skin
High level of LDH
Findings against congenital leukemia
Borderline low hematocrit of 41%
Markedly high platelet count of 949,000
Transient myeloproliferative disorder associated with mosaicism for trisomy 21- Natural History of the Disorder
Pediatric Oncology Group carried out a prospective study to determine the natural history of the condition
48 infants with trisomy 21 and transient myeloproliferative disorder were followed
Median age of diagnosis was 7 days
25% were entirely asymptomatic
6 of the children had a rash, 2 similar to the patient in this case
41 of the 48 had spontaneous clearance at a median of 1 month
8 died from either liver failure or multi-organ failure and sepsis
