Case_1-2004

Immunology Cases 2004 Case 1 W.Y. is a 19-month-old male who presents with fever. He was well until 5 months of age when he presented with his first episode of pneumonia. He had one additional episode of pneumonia and three episodes of otitis media. 1 In the Emergency Room, his vital signs were: T 104°F, pulse 140, respiratory rate 26, and BP 150/90. He appeared small for his age. The remainder of his physical exam was notable for bulging tympanic membranes and râles 2 over the left lower lung field. Laboratory examination was notable for leukocytosis 3 to 18 4 with 90% polys 5 (normal 40- 70%), 7% bands 6 (normal 3-5%), and 3% lymphocytes (normal 10-50%). Chest X-ray showed a dense infiltrate in the left lower lobe. 7 The patient was admitted for treatment of pneumonia and otitis media. In the hospital, additional testing was performed to investigate the reason for this child’s frequent bacterial infections. An HIV test was negative. RBC adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP) were normal. Serum protein electrophoresis showed decreased gamma globulins and quantitative serum immunoglobulins showed an IgM of 20 mg/dl, IgG 50 mg/dl, and IgA of 10 mg/dl (normal values are IgG > 800, IgA > 50, and IgM > 40 mg/dl). Analysis of the child’s peripheral blood lymphocytes by flow cytometry showed that they were all T-cells. 1 Middle ear infection. Although this is often viral in etiology, the three most common bacterial etiologies are Streptococcus pneumoniae (pneumococcus), Haemophilus influenzae and Moraxella catarrhalis. 2 Velcro-like lung sounds, usually upon inspiration. This physical sign is non-specific, but is typical of pulmonary edema (excess lung water) or consolidation (e.g., reflecting the exudate that accompanies bacterial pneumonia). 3 Elevated white blood cell (WBC) count 4 In case presentations, units for many values are often omitted. In this case “18” refers to 18 X 10 9 /liter, which is equivalent to 18,000 cells/μl. 5 A common abbreviation for polymorphonuclear leukocytes, or neutrophils 6 Bands are immature neutrophils whose nuclei appear “band-like” rather than multi-lobed. This is indicative of rapid production and egress of neutrophils from the bone marrow, typically stimulated by infection. 7 Synonymous terms include “left lower lobe (LLL) consolidation.” On chest radiographs, this appears as confluent shadows in the left lower lobe, which is indicative of an alveolar filling process, such as bacterial pneumonia. Questions for Case 1 (1) What disease does this child have? How do the cells and molecules absent/diminished in this patient normally protect an individual from infection? (2) What types of infections would this child be expected to have frequently? What types of infections should not pose a serious threat to this child? (3) Why did this child not have any infections during his first few months of life? (4) At what stage in development are this patient’s B-lineage lymphocytes arrested? How might you attempt to demonstrate this in the laboratory? (5) All the peripheral blood lymphocytes were T-cells. What cell surface markers might have been used to confirm this? (6) Describe known genetic defects that lead to hypogammaglobulinemia. (7) What therapeutic options are available for this patient?

Upload
andrespimentelalvarez
Category

Documents
view
215
download
2

Embed Size (px):

description

Describe un caso clinico de inmunologia que sdebe ser resuelto consultando el libro de Janeway

Transcript of Case_1-2004

Immunology Cases 2004

Case 1

W.Y. is a 19-month-old male who presents with fever. He was well until 5 months of age whenhe presented with his first episode of pneumonia. He had one additional episode of pneumoniaand three episodes of otitis media.1 In the Emergency Room, his vital signs were: T 104F, pulse140, respiratory rate 26, and BP 150/90. He appeared small for his age. The remainder of hisphysical exam was notable for bulging tympanic membranes and rles2 over the left lower lungfield. Laboratory examination was notable for leukocytosis3 to 184 with 90% polys5 (normal 40-70%), 7% bands6 (normal 3-5%), and 3% lymphocytes (normal 10-50%). Chest X-ray showed adense infiltrate in the left lower lobe.7 The patient was admitted for treatment of pneumonia andotitis media. In the hospital, additional testing was performed to investigate the reason for thischilds frequent bacterial infections. An HIV test was negative. RBC adenosine deaminase(ADA) and purine nucleoside phosphorylase (PNP) were normal. Serum protein electrophoresisshowed decreased gamma globulins and quantitative serum immunoglobulins showed an IgM of20 mg/dl, IgG 50 mg/dl, and IgA of 10 mg/dl (normal values are IgG > 800, IgA > 50, and IgM> 40 mg/dl). Analysis of the childs peripheral blood lymphocytes by flow cytometry showedthat they were all T-cells.1Middle ear infection. Although this is often viral in etiology, the three most common bacterial etiologies areStreptococcus pneumoniae (pneumococcus), Haemophilus influenzae and Moraxella catarrhalis.2Velcro-like lung sounds, usually upon inspiration. This physical sign is non-specific, but is typical of pulmonaryedema (excess lung water) or consolidation (e.g., reflecting the exudate that accompanies bacterial pneumonia).3Elevated white blood cell (WBC) count4In case presentations, units for many values are often omitted. In this case 18 refers to 18 X 109/liter, which isequivalent to 18,000 cells/l.5A common abbreviation for polymorphonuclear leukocytes, or neutrophils6Bands are immature neutrophils whose nuclei appear band-like rather than multi-lobed. This is indicative of rapidproduction and egress of neutrophils from the bone marrow, typically stimulated by infection.7Synonymous terms include left lower lobe (LLL) consolidation. On chest radiographs, this appears as confluentshadows in the left lower lobe, which is indicative of an alveolar filling process, such as bacterial pneumonia.

Questions for Case 1

(1) What disease does this child have? How do the cells and molecules absent/diminished in thispatient normally protect an individual from infection?

(2) What types of infections would this child be expected to have frequently? What types ofinfections should not pose a serious threat to this child?

(3) Why did this child not have any infections during his first few months of life?

(4) At what stage in development are this patients B-lineage lymphocytes arrested? How mightyou attempt to demonstrate this in the laboratory?

(5) All the peripheral blood lymphocytes were T-cells. What cell surface markers might havebeen used to confirm this?

(6) Describe known genetic defects that lead to hypogammaglobulinemia.

(7) What therapeutic options are available for this patient?