Neutrophil and platelet disorders

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4.2 2017
Averil Tam
Fichas por Averil Tam, actualizado hace más de 1 año
Averil Tam
Creado por Averil Tam hace más de 6 años
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Resumen del Recurso

Pregunta Respuesta
1. YES/NO: The following is concerning and requires further Ix/review: A) 4 month old girl with a neutrophil count of 0.9 x 10^9/L No, within normal limits
1. YES/NO: The following is concerning and requires further Ix/review: B) 2 year old boy with recurrent otitis media and a neutrophil count varying between 0.2–1.0 x 10^9/L Yes, may have autoimmune neutropaenia or cyclic neutropaenia
1. YES/NO: The following is concerning and requires further Ix/review: C) 10 year old girl from Sudan with a neutrophil count of 1.0 x 10^9/L No, consistent with benign ethnic neutropaenia
1. YES/NO: The following is concerning and requires further Ix/review: D) 15 year old boy with neutrophil count of 0.6 x 10^9/L after rituximab for renal disease Yes, may be late onset drug induced neutropaenia
2. 11 month old twins, previously well and thriving. 6 weeks of recurrent skin infections now growing pseudomonas. Apart from the skin lesions, exam normal. Both neutropenic (0.1 x 10^9/L). Bone marrow examination: normocellularity with increased myeloid activity with a left-shift and with reduction in mature forms; small amount of neutrophil phagocytosis; normal erythroid and megakaryocytic activity, no evidence of leukaemia. Most likely Dx: A. Severe congenital neutropenia B. Alloimmune neutropenia C. Autoimmune neutropenia of infancy D. Hypersplenism C, most likely diagnosis is autoimmune neutropenia. The bone marrow findings of good myelopoiesis are against a diagnosis of SCN. Too old to have alloimmune neutropenia (usually resolves by 3-4 months). BM findings are typical of autoimmune neutropenia (including neutrophil phagocytosis). No evidence of splenomegaly to support hypersplenism.
3. Which of the following features is most consistent with a diagnosis of ITP A) Absence of dysmorphic features B) Elevated MCV for age C) History of easy bruising since infancy D) Reduced Hb for age E) Presence of hepatosplenomegaly A, all the rest are wrong. Since ITP is a diagnosis of exclusion the presence of any of these features suggest alternative diagnoses that must be looked for.
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