Michael B. Harris, MD Co-Director, Institute for Pediatric Cancer and Blood Disorders Chief, Pediatric Hematology/Oncology Joseph M.

Transcription

1 Michael B. Harris, MD Co-Director, Institute for Pediatric Cancer and Blood Disorders Chief, Pediatric Hematology/Oncology Joseph M. Sanzari Children s Hospital Hackensack University Medical Center

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3 Presented to pediatrician with mild cough Without fever, skin rash or acute infection and no history of chronic diaper rash, serious infections (ex. sepsis, pneumonia), or repetitive infections (ex. recurrent OM, skin infections) or stomatitis Birth history and neonatal course are normal No family history of neutropenia or other blood disorders Had routine CBC prior to presentation to PCP WBC 12,400 with ANC of 100 Hgb and platelet count are normal What is the most likely diagnosis?

4 The mechanism of neutropenia Destruction or failure of normal production Severe clinical neutropenia usually is a failure of production Chronic benign neutropenia is secondary to destruction Congenital neutropenias are a failure of normal myelopoeisis: some are severe (Kostmann), moderate to mild (cyclic neutropenia) to benign familial neutropenia. Immune neutropenias are usually self-limited with mild clinical courses

5 Age at diagnosis Antecedent infections viral, bacterial/fungal History of infections Frequency Severity Location Drug history prior to diagnosis Associated illnesses autoimmune disorders Neonatal history Family history

6 Neonate to first year of life Alloimmune Severe congenital neutropenia Cyclic neutropenia (may not be diagnosed until later) Familial Neutropenias (Kostmann, Scwachman- Diamond, etc.) 3-38 months Chronic benign/autoimmune neutropenia of childhood Viral induced All ages AIN (secondary to autoimmune disease, drugs, viral infections), familial neutropenia, ethnicity

7 Neutropenia ranges from 10-30% of population Africans African-Carribean West Indies Ethiopians Yemenite Jews Arabs Clinical features CBC is normal other than neutropenia Without history of local or systemic infections

8 Data collected from the National Health and Nutritional Examination Survey (NHANES) Variables were ethnicity, sex, age and smoking status Population studied: Blacks, Mexican Americans and Whites 25,222 of 29,608 individuals without missing data were analyzed 3 ml of blood drawn for CBC Blood drawn for a one time analysis Hsieh et al. Ann Intern Med. 2007;146(7):

9 From: Prevalence of Neutropenia in the U.S. Population: Age, Sex, Smoking Status, and Ethnic Differences Ann Intern Med. 2007;146(7): doi: / Figure Legend: Distribution of leukocyte (top) and neutrophil (bottom) counts in persons age 18 years or older from 3 ethnic groups.appendix Table 3 shows the actual percentages and number of participants. Date of download: 4/25/2014 Copyright American College of Physicians. All rights reserved.

10 From: Prevalence of Neutropenia in the U.S. Population: Age, Sex, Smoking Status, and Ethnic Differences Ann Intern Med. 2007;146(7): doi: / Figure Legend: Percentage of females (top) and males (bottom) with neutrophil counts less than cells/l.the error bars refer to the standard errors. Appendix Table 4 shows the actual percentages and number of participants. Date of download: 4/25/2014 Copyright American College of Physicians. All rights reserved.

11 One week after presentation ANC = 300 Ten days after presentation febrile illness with cough and ANC =1400 One month later ANC = 2500 Most likely diagnosis Viral induced neutropenia Viral illness may be subclinical or mild Recovery is usually fairly quick No history of previous severe infections, stomatitis, etc.

12 Incidence: 1per 100,000 Age at presentation 3 to 38 months Median age: 8-11 months Relatively minor (gingivitis, skin, otitis media, URI) to no infections; however, occasional serious infections in some patients (pneumonia) ANC at diagnosis Antibodies such as anti-na1 may be found Bone marrow (if done) shows increase in myelopoiesis, occasional decrease in mature neutrophils Dinauer M, Newburger P in Hematology of Infancy and Childhood (2009); Orkin et al (ed)

13 Therapy Antibiotics for acute infections Prophylactic antibiotics for patients with recurrent infections such as otitis media G-CSF in event of serious infections or very high frequency of recurrent, minor infections Prognosis Excellent Median time to recovery from diagnosis is twenty months (6-54 months) Dinauer M, Newburger P in Hematology of Infancy and Childhood (2009); Orkin et al (ed)

14 Born with normal CBC (will most probably not have this result) Benign neonatal course without severe infections, recurrent fevers, stomatitis, chronic diaper rash, thriving Well appearing child with normal physical exam Development after a probable viral illness CBC normal except for low ANC

15 When in doubt always call

16 Thank you