RHINOSCLEROMA NOTES

 

INTRODUCTION

Chronic granulomatous infectious disease primarily affects upper respiratory tract.

 

EPIDEMIOLOGY

 

Affects all ages-young, first 3 decades, world wide distribution ,  low socioeconomic background with poor living conditions , malnutrition.

HISTORICAL IMPORTANCE

• For the first time VON FRISH identified the causative organism of the disease RHINOSCLEROMA.
• MIKULICZ has described the classical FOAMY cells in the histopathology of            RHINOSCLEROMA. 
• VON HERBA discovered the disease , after his name HERBA NOSE.
• VON MIKULICZ studied the histopathology of disease.

CAUSATIVE AGENT

KLEBSIELLA RHINOSCLEROMATIS ( Frisch bacillus ) – 2.5 um , capsulated ,gram negative , immotile organism belongs to ENTEROBACTERIACEAE FAMILY.

ROUTE OF TRANSMISION

Through droplet spread.

 

LOCALIZATION

Nasal cavity

Palpebral conjunctiva

Middle ear

Nasopharynx

Tracheobronchial tree

Esophagus

Larynx

Pharynx

DIAGNOSIS

Biopsy of the involved site , demonstration of bacilli with special stains ,supplemented by immunohistochemistry.

 

IMAGING

• Intracranial extension of the disease due to destruction of posterior wall of left sphenoidal         sinus by CT SCAN.
• MRI detects nasal mass extending into nasopharynx.

GROSS PATHOLOGY

Infected mucosa is pale in appearance & demonstrates diffuse nodular thickening(FLORID PHASE).

 

MICROSCOPY

1.Squamous metaplasia with inflamed granulation  tissue is seen in early stages of the disease.

 

2.FLORID/PROLIFERATIVE PHASE:

submucosal granulomatous infiltrate composed of macrophages with clear foamy cytoplasm (MIKULICZ CELLS) intimately associated with admixture of lymphocytes & numerous plasma cells.rod shaped gram negative organisms can be demonstrated in MIKULICZ cells.

3.FIBROTIC/RESOLUTION PHASE: 

no Mikulicz cells , extensively replaced by fibrosis and lymphocytes and plasma cells (russel bodies – accumulation of immunoglobulins).

HIGH POWER IMAGE SHOWA MIKULICZ CELLS WITH ABUNDANT FOAMY CYTOPLASM.

 

SPECIAL STAINS

GIEMSA , WARTHIN STARRY STAIN, Dieterle stain    & PAS positive (inclusion bodies ).

IMMUNOHISTOCHEMISTRY

MIKULICZ CELLS-CD68 positive.

Langerin,S100,CD1a negative.

ELECTRON MICROSCOPY

Bacilli and granular material is seen in large phagosomes.

 

DIFFERENTIAL DIAGNOSIS

Syphilis- plasma cells ,detection of Treponema pallidum by dark field microscopy.

Rosai dorfmann disease - sinus histiocytosis with massive lymphadenopathy , emperiploesis.

Inflammatory pseudotumour/plasma cell granuloma-along with mixed inflammatory infiltrate , plasma cells , vascular proliferation there is storiform pattern of arrangement of fibroblasts/myofibroblasts.

Wegners granulomatosis( triad of focal glomerulonephritis , vasculitis and necrosis of Upper respiratory tract) now named as GRANULOMATOSIS WITH POLYANGIITIS.

Malignant lymphoma - clonal disorder , absence of mikulicz cells , Immunohistochemistry helps in doubtful cases.

MANAGEMENT

Long term antibiotics followed by surgical debridement

Surgical resection

Co2 laser surgery

OUTCOME

Slowly progressive disease with relapses and recurrences.

Fatal if obstructs trachea.

 

KIMURA DISEASE

NTRODUCTION

A chronic inflammatory disorder  of uncertain etiology with distinctive histologic features mainly affecting the head and neck that appears to represent an aberrant immunologic reaction characterized by eosinophilia , increased serum IgE and angiolymphoid proliferation.

SYNONYMS

Kimura lymphadenopathy , Eosinophilic lymphogranuloma

INCIDENCE uncommon

GENDER,RACE, AND AGE DISTRIBUTION

Most patients are young adult males, Asians are more commonly affected than whites or blacks.

ETIOLOGY –unknown.

 

CLINICAL FEATURES

Patients typically have  painless unilateral or bilateral cervical lymphadenopathy.

One or more  slowly growing , large , painless lesions in subcutaneous and deep soft tissue which may persist or recur over a period of months or years.

Some patients may have proteinuria , nephrotic syndrome or asthma.

MORPHOLOGY

Lesions generally poorly demarcated.

Lymph nodes and other involved sites show an infiltrate of lymphocytes, eosinophils, plasma cells, mast cells, reactive follicles, and fibrosis.

Eosinophilic abscesses and polykaryocytes, especially in follicles.

Collagen deposition may be seen , on long standing  diffuse hyalinization changes may be seen.

EXTENSIVE SUBCUTANEOUS INVOLVEMENT BY KIMURA DISEASE IN A YOUNG MALE - LOW POWER VIEW

SCANNER VIEW SHOWING REACTIVE FOLLICLES WITH EOSINOPHILS WITHIN THEM.

LOW POWER VIEW SHOWING HYPERPLASTIC FOLLICLE WITH NUMEROUS EOSINOPHILS WITH IN AND AROUND THE FOLLICLE

HIGH POWER VIEW SHOWING PREDOMINANCE OF EOSINOPHILS IN KIMURA LYMPHADENOPATHY

IMMUNOPHENOTYPE

Ig E positive follicular dendritic networks in follicles.

Vascular endothelial cells – factor VIII , Ulex europaeus agglutinin (UEA-1).

DIAGNOSIS

Biopsy of involoved tissue

DIFFERNETIAL DIAGNOSIS

 

PROGNOSIS AND THERAPY

The lymphadenopathy and other mass lesions do not require specific therapy , although large and persistent can be excised.

 

CONCLUSION

Chronic inflammatory disease of uncertain etiology , commonly seen in young Asian males with involvement of lymphnodes , salivary glands and kidneys, hyperplastic germinal centers with abundant eosinopilic infiltration  involving germinal centers and microabscess ,peripheral blood eosinophilia and increased IgE levels.

EXTRA EDGE

https://youtu.be/mhsNWo6Lwm8I

KIKUCHI FUJIMOTO DISEASE

DEFINITION

Benign disease  characterized by lymphadenopathy with distinctive  histological features.

SYNONYMS :

INCIDENCE   Worldwide distribution, however high incidence in Asians.

EPIDEMIOLOGY -  Most patients are  young women , the average age at presentation 27years.

ETIOLOGY – unknown.

CLINICAL FEATURES -  Unilateral tender post cervical lymphadenopathy, fever, rash, anemia, and leukopenia. rarely presents with the atypical lymphocytosis.

MORPHOLOGY:

• Patchy Lymph node involvement , usually centered  in paracortical areas
• Atleast occasional crescentic histiocytes
• Fibrinoid necrosis surrounded by immunoblasts , histiocytes and plasmacytoid dendritic cells.
• Extracellular apoptotic/karyorrhectic debri
• Absence of eosinophils,polymorphonuclear cells and plasma cells

EXTENSIVE LYMPH NODE INVOLVEMENT WITHOUT EFFACEMENT OF LYMPH NODE ARCHITECTURE AND RESIDUAL FOLLICLES (SCANNER VIEW).

PROLIFERATIVE TYPE WITHOUT NECROSIS (LOW POWER)

NECROTIZING TYPE WITH ABUNDANT NECROTIC FOCI ( LOW POWER VIEW )

HIGH POWER VIEW SHOWING ABUNDANT KARYORRHECTIC DEBRI WITH ABSENCE OF NEUTROPHILS

HIGH POWER VIEW SHOWING CRESCENTIC HISTIOCYTES

HISTOLOGIC SUBTYPES :

IMMUNOPHENOTYPE

plasmacytoid dendritic cells express CD 2 , CD4 , CD 43 , CD68  and CD123.

DIAGNOSIS

LYMPH NODE BIOPSY

DIFFERNTIAL DIAGNOSIS

PROGNOSIS

Some  patients develop recurrent lymphadenopathy

Rare patients, usually with an underlying immunodeficiency , have a severe illness with a poor outcome.

MANAGEMENT- nearly all patients have a self limited illness requiring no specific therapy. 

https://youtu.be/nxlCK6wkOmQhttps://youtu.be/nxlCK6wkOmQ

https://youtu.be/nxlCK6wkOmQhttps://youtu.be/nxlCK6wkOmQ

HEREDITARY SPHEROCYTOSIS

 INTRODUCTION

HS is one among the foremost common Hemolytic anemias, with a frequency of one in 5000 within the U.S. and one in 2000 in people of northern European ancestry, conjointly seen in southeast Asia, India, and the Kingdom of Nepal.

DEFINITION

Hemolytic Disorder is caused by defective vertical interactions between the lipid bilayer and body structure of the red blood cell membrane, leading to vesication and loss of membrane, red blood cell formation, and lysis (spherocytes are less deformable than a traditional red blood cell, trapped  and destroyed within the spleen.)

INHERITANCE-AD most typical, AR inheritance is seen in few cases.

INCIDENCE - One in 5000 within the U.S.

GENDER, RACE AND AGE DISTRIBUTION - Equal gender incidence , MC in patients of northern European descent (1 in 2000).

CLINICAL FEATURES - TRIAD OF Variably severe hemolytic anemia, jaundice, spleenomegaly. 

typically follows a stable clinical course, however could also be punctuated by

a)APLASTIC CRISIS- triggered by acute parvo infection.

b)HEMOLYTIC  CRISIS – intercurrent events resulting in raised RBC destruction like in glandular fever.

Cholelithiasis happens in 40-50% of patients.

Other presentations- hemolytic disease  of newborn and HS in pregnancy.

CLINICAL CLASSIFICATION based on  SPECTRIN CONTENT

MILD HS:80-100%

MODERATE HS: 50-80%

MODERATELY SEVERE HS: 40-70%

SEVERE HS: 20-50%

PATHOGENESIS:

LIFGHT MICROSCOPY :

PERIPHERAL BLOOD SMEAR-Spherocytes ( dark red  with no central pallor), polychromasia.

INCREASED retic count ( 5-40%).

SPLEEN- engorged splenic  cords, increase in range of splenic macrophages with phagocytosed red cells in them.

BONE MARROW –erythroid hyperplasia with normocytic reaction.

PERIPHERAL BLOOD FILM SHOWING SPHEROCYTES

ANCILLARY STUDIES

The screening tests –

a)OSMOTIC FRAGILITY –spherocytes are osmotically fragile.

b)EOSIN five MALEIMIDE – sensitive screening test –decreased fluorescence is seen in Hereditary Spherocytosis.

Others tests- incubated fragility test, glycerol lysis test, ektacytometry .

Biochemistry –serum bilirubin, urine bilirubin, urine urobilinogen raised.

Serum haptoglobin levels reduced. 

serum LDH levels raised.

Direct coombs test negative.

Definitive diagnosis – distinguishing the defective protein by SDS PAGE  ANALYSIS OR  DNA SEQUENCING.

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS  

COMPLICATIONS

PROGNOSIS AND THERAPY

Supportive care, no specific medical aid.

Severe cases- folic acid supplementation and SPLEENECTOMY.

MORBIDITY AND MORTALITY

Mild morbidity due to complications of chronic hemolysis.

Aplastic crises due to parvo B19

Megaloblastic anemia

Slightly raised mortality, typically a result of post splenectomy infection.