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We report herein a case of isolated Rosai-Dorfman disease (Sinus Histiocytosis with Massive Lymphadenopathy) diagnosed upon routine microscopic examination of tonsils in a child. The patient is a 4 year old girl who had a history of sleep apnea, but no other significant medical history or other findings on physical examination. In this report, we discuss the various approaches to handling of tonsillectomy specimens. We also discuss the value of routine microscopic examination, providing a list of case examples of incidental, clinically important, findings that can be missed if a microscopic examination of tonsils was not performed. We finally discuss the clinical significance of finding histologic evidence of Rosai-Dorfman disease in a child.
Hemoglobin K-Woolwich (Hb KW)
is a rare hemoglobin variant with very few cases reported. It is most prevalent
in West African countries, particularly Nigeria, Ghana, and the Ivory Coast.
Some reports suggest Hb KW may be a clinically benign trait, whereas others
indicate it may behave similarly to a β+
thalassemia. The combination of hemoglobin S and hemoglobin KW (Hb S/KW) is a
rare double heterozygous disorder with little known clinical characteristics.
We report the hematologic and clinical data on three patients with Hb S/KW to
help describe the characteristics of this patient population. The first two
cases represent first cousins, ages 3 and 2 years. They are clinically
asymptomatic. They have normal hemoglobin and mean corpuscle volume (MCV)
levels without reticulocytosis. The third case is of a 14-year-old male who is
non-anemic with no microcytosis. He has been clinically well except for
abdominal pain upon dehydration. On hemoglobin electrophoresis, these patients
have Hb S levels slightly higher than typically observed with sickle cell trait
and a delay of hemoglobin F to adult levels. There exists a need for more
reports to better delineate the clinical course and management of these
Neutropenia in a child presents a wide differential diagnosis, spanning the
spectrum of benign disorders (such as congenital neutropenia, cyclic
neutropenia, etc.) to malignant processes (such as myelodysplasia or acute
leukemia). The morphologic findings in the bone marrow of a patient with neutropenia
may show considerable overlap with those seen in hematopoietic malignancies. We
present herein a case that demonstrates this overlap and discuss features that
can help in avoiding diagnostic pitfalls.