Bilobed or dumbbell-shaped neutrophils with more condensedĬhromatin (nuclear material) are seen. The condition is diagnosed by observation of white cells in a blood Related dogs with the condition Diagnostic Tests The responsible gene has not yet been identified In humans, mutations in the Lamin B Receptor (LBR) gene, which codes forĪ protein in the nuclear membrane, have been identified as being Lower litter sizes surviving to weaning than in normal dogs.If a defective gene is inherited from each parent (homozygous), theĭisorder in dogs is typically lethal in utero or shortly after birth, PHA can exist by itself with no detrimental effect to the dog. When only one defective copy of the gene is inherited (heterozygous), Samoyeds, white cell changes similar to PHA were reported along withĭwarfism and ocular problems.) Signs and Symptoms Gene) it is associated with skeletal abnormalities.
When homozygous in humans (two copies of defective In other species it can be associated withĪdditional problems. Reported in humans, rabbits, mice and domestic short-haired cats. (dog may have defective gene without exhibiting the disorder). It is also a known problem in Australian Shepherd dogs,Īlthough in this breed the penetrance of the trait appears incomplete Has been reported to occur in foxhounds, Samoyeds and other dogs, e.g.īasenji. Related to sex of animal, and one copy of gene leads to disorder) that The disorder appears to be an autosomal dominant genetic trait (not The nuclei of several types of white blood cells (neutrophils andĮosinophils) have unusual shape (bilobed or dumbbell-shaped) and
Pelger-Huët anomaly (PHA) is a rare, inherited blood condition in which If you would like to volunteer, please contact and we will be happy to answer any of your questions.įor a description of the position, please click on disorder health information liaison. Follow-up visit in the clinic subsequently demonstrated resolution of the anomaly and normal neutrophils.Note: We do not currently have a health liaison for this disorder. Ibuprofen was discontinued as it was deemed as the likely culprit causative agent for acquired PHA. These findings were consistent with benign heterozygous PHA. The remaining 80% of the neutrophils showed bi-lobed nuclei without a thin filament that made them look like band cells. 20% of the neutrophils have bi-lobed nuclei with a thin filament connecting the lobes. A peripheral blood smear showed normal mature chromatin condensation and granulation. Over the next two days the patient remained afebrile and had no new signs or symptoms. Urinalysis and chest x-ray did not show any evidence of infection. Vital signs were within normal limits Laboratory tests on presentation were significant for a normal white blood cell (WBC) count with elevated band forms (24%), ESR (38), and CRP (7.3). On examination, the patient had thick hands without an obvious joint swelling. Patient's home medications included Ibuprofen 600 mg QID, methotrexate and hydroxychoroquine. The past medical history was significant for rheumatoid arthritis and diabetes mellitus type 2. CASE PRESENTATION: A 67 year old gentleman presented with a 1-day history of fever and night sweats.
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