ID Clinical Case Conference

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ID Clinical Case Conference

• Munshi Moyenuddin, M.D.
• Fellow, Infectious Diseases
• WFUBMC
• 25 November, 2002

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Case1 40 y/o AAM with neck mass

• The pt presented to PCP with an increasing mass
  on the L-side of his neck for 3-wks.
• Initially the mass was noticed by his wife.
• He denied any trauma, fever, pain, dysphagia,
  cough, SOB, or other complaints

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HP (contd)

• PMH Parotid gland swelling- 6 m ago, Peptic
  ulcer disease- 3 y ago, h/o gonorrhea- 10 y ago.
• PSH Adenoid surgery- 1 y ago
• Allergy None
• Meds None
• SHX Married for 8 years, 2-children, works in
  Fedex, denied tobacco/ ETOH/ drugs, heterosexual,
  lives with wife

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CT Scan of neck

• Extensive adenopathy throughout all of the
  triangles of neck, extending down to the
  supraclavicular fossa.
• The nodes measure about 3 cm in dimensions,
  confluent in some areas
• Parotid glands were enlarged bilaterally and
  contain small lucent areas suggesting cystic
  transformation.
• Findings raise question of systemic reactive or
  lymphoproliferative disease.

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Disease Course

• T-97.7, P-93, R- 23, BP-153/86
• Gen wellnourished male, NAD
• Neck- 5x5 cm mass on the L-neck, irregular
  surface, cystic anteriorly, firm posteriorly,
  nontender, nonmobile, no erythema
• Asymmatric swelling of the parotid glands RL
  nontender
• Skin no rash
• Heart/Lung/Abdomen unremarkable

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Labs

• FNA of a L-neck lymph node findings suspicious
  for Hodgkin lymphoma but need additional tissue
  for definitive diagnosis.
• Wbc- 4.0, Hg- 12.0, MCV- 89.6, Plt- 272, seg-
  61, lymph- 32 (ALC 1300)
• Na- 134, K- 4.0, HCO3- 26, BUN- 15, Cr- 1.1, LFT-
  nml
• ESR- 101

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Labs (contd)

• HIV Ab- positive
• CD4- 220, VL- 25,655
• Hep A Ab- neg, HBsAb- neg, HBcAb-neg, Hep C Ab-
  neg
• RPR- NR, Toxo IgG- 0, CMV IgG- 99
• Lymphnode Bx Follicular paracortical
  hyperplasia, plasmacytosis, focal follicular
  lysis and sinus histiocytosis. The features are
  consistent with a reactive proliferation seen in
  HIV-associated lymphadenopathy

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Disease Course

• The pt was started on HAART with good compliance
  and tolerance
• Neck mass decreased in size
• Pt remained asymptomatic
• 2-m follow up visit in clinic is pending

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Head and neck mass in HIV infection (J Laryng
Otol 1993 107133)

• From 1987 to 1991, 210 HIV pts were studied for
  frequency of enlarged neck nodes, neck mass,
  nasopharyngeal lymphatic adenopathy,
  non-hodgkins lymphoma, kaposis sarcoma, and oral
  hairy leukoplakia.
• 84 of the pts had head and neck manifestations.
• Neck lymphadenopathy (1-3 cm) was observed in 19
  of the asymptomatic pts and 38 of the pts with
  persistent generalized lymphadenopathy
  (lymphadenopathy 1cm at 2 or more extra inguinal
  sites lasting 3m without another etiology)

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Head and neck mass in HIV (contd)

• Neck mass 3 cm were observed in 3- 17 in
  different subgroups
• Another study also indicated that enlargement of
  neck nodes was common among HIV pts (24) (Head
  and Neck 199113522).
• The lymphatic tissue within the parotid gland may
  also be the target of HIV causing dilatation of
  the salivary ducts and production of
  lymphoepithelial cyst (Head and Neck 199012337)
• The extranodal nasopharyngeal area shows
  hypertrophy during the early stages and a later
  reduction (Arch Otolaryng 1990116928)

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Parotid gland swelling in HIV

• Two case reports with bilateral parotid gland
  swellings described the phenomenon as diffuse
  infiltrative CD8 lymphocytosis syndrome (Oral
  Surg Oral Med Oral Pathol Oral Radiol Endod 1998
  85 565).
• Initially the glandular enlargement resulted from
  a massive CD8 cell lymphoproliferation,
  subsequently, lymphoepithelial cysts were
  developed.

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Parotid gland swelling in HIV (contd)

• The characteristics of diffuse infiltrative
  lymphocytosis syndrome (DILS) include (1)
  persistent circulating CD8 lymphocytosis, (2) CD8
  lymphocytic tissue infiltration with generalized
  lymphadenopathy, and (3) parotid gland
  enlargement.
• DILS commonly involves the salivary glands and
  the lungs and less frequantly, involves the
  liver, kidney, gastrointestinal tract, muscle,
  and peripheral nerve system (Ann Intern Med
  19901123 Ann Neurol 1997 41 438 AIDS 1996
  10 385).

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Parotid gland swelling in HIV (contd)

• The natural history of parotid lymphocytic
  hyperplasia is not fully known.
• Pts with DILS appear to be at significantly
  increased risk of salivary gland B-cell lymphoma
  (Rheum Dis Clin North Am 199218683).
• Oral prednisone or HAART with zidovudine (or
  both) offers the best means of eliminating the
  parotid swellings (AIDS 199610385).
• If there is no indications of malignancy,
  observation with periodic FNA monitoring is all
  that is required.

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Chronic hypertrophic parotitis in HIV (Rom J
Virol 199546135)

• A 3-years study in hospitalized HIV positive
  children (2-15 y) showed chr hypertrophic
  parotitis (uni or bilateral) in 23.3.
• Anti-CMV IgG was in 41 and anti- toxo IgG was
  in 50.
• Significant increase in the level of
  immunoglobulins (IgG- 92, IgM- 85) was noted in
  these pts.
• CHP appeared in most pts (67) before a marked
  deterioration of CD4, CD8 were frequently
  increased (94)

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HIV Lymphadenitis

• 3 histological patterns, A, B, and C, have been
  described that generally correspond to clinical
  stages of acute, chronic, and burnout (NEJM 1993
  328 327 Am J Surg Pathol 199620572).
• Pattern A shows greatly enlarged lymphoid
  follicles comprising of reactive hyperplastic
  germinal centers with widespread apoptosis,
  phagocytosis of nuclear debries by histiocytes
  small lymphocytes penetrate in the germinal
  centers contribute to disruption (folliculolysis)

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HIV lymphadenitis (contd)

• Pattern B is a transition from pattern A to
  pattern C. It includes effacement of follicles,
  disruption of dendritic cells, and involution of
  germinal centers. They are suggestive of subacute
  or chronic lymphadenitis.
• Pattern C shows lymphnodes with atrophic,
  burned-out follicles and extensive, diffuse
  vascular proliferation. The interfollicular
  cortex shows loss of lymphocytes but plasma cells
  and fibrosis are seen.

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HIV Lymphadenitis (contd)

• In a study of HIV-lymphadenitis 79 pts were
  followed for 7 years of 31 pts who initially
  showed the histologic A-pattern, 18(58) remained
  stationary and 13 (42) progressed to AIDS
• Of 31 pts with a B-pattern, 11 (36) remained
  stationary and 20 (64) progressed to AIDS
• Of 17 pts with a C-pattern, 1 (6) remained
  stationary and 16 (94) progressed to AIDS

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Differential Diagnosis of HIV Lymphadenitis

• Pattern A (acute)
• Infectious mononucleosis lymphadenitis
• Cytomegalovirus, varicella, measles lymphadenitis
• Toxoplasma lymphadenitis
• Pattern B (chronic)
• Castleman lymphadenopathy, plasma cell type
• Angioimmunoblastic lymphadenopathy

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Diff Dx of HIV lymphadenitis (contd)

• Pattern C (burnout)
• Castleman lymphadenopathy, hyaline vascular type
• Fibrosed end-stage lymphadenitis