Hodgkin lymphoma

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Classical Hodgkin lymphoma
Diagnosis in short

HL mixed cellularity - cytology.

LM Reed-Sternberg cell (large binucleated cell (>= 45 micrometres), +/-multinucleated, +/-horseshoe-like shape, macronucleolus - approximately the size of a RBC (~8 micrometers)), well-defined cell border, abundant cytoplasm.
Subtypes nodular sclerosis CHL, mixed cellularity CHL, lymphocyte-rich CHL, lymphocyte-depleted CHL
LM DDx

diffuse large B cell lymphoma (esp. T-cell/histiocytic-rich LBCL), anaplastic large cell lymphoma,

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma, nodular lymphocyte-predominant Hodgkin lymphoma
IHC CD30 Reed-Sternberg cells (RSCs) +ve ~98%, CD15 Reed-Sternberg cells +ve ~80% (also stains neutrophils), CD45 often negative in RSCs, CD20 -ve/+ve, PAX5 +ve
Site usu. lymph node - classically in the neck

Clinical history young adults, older adults (bimodal distribution)
Signs lymphadenopathy - usu. neck, +/-B symptoms (fever, night sweats, weight loss)
Prevalence common
Prognosis usu. good, dependent on stage
Nodular lymphocyte-predominant Hodgkin lymphoma
Diagnosis in short

Popcorn cell in nodular lymphocyte-predominant Hodgkin lymphoma

LM Popcorn cells (relatively) small (compared to classic RSCs) - have lobulated nucleus (key feature), small nucleoli; subtle nodularity at low power
Subtypes none
LM DDx

diffuse large B cell lymphoma (esp. T-cell/histiocytic-rich LBCL), anaplastic large cell lymphoma,

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma, classical Hodgkin lymphoma
IHC LCA (CD45) +ve, CD20 +ve, CD10 +ve, Bcl-6 +ve, EMA +ve, CD30 -ve, CD15 -ve
Site usu. lymph node

Signs lymphadenopathy
Prevalence uncommon
Prognosis good

Hodgkin lymphoma, abbreviated HL, is a malignancy that often afflicts people in the prime of their life. Fortunately, it usually has a good prognosis.

Pathologists say "... it is both the easiest and hardest diagnosis to make." The reason for this is: the diagnosis depends on finding Reed-Sternberg cells (or Popcorn cells); if they are obvious the diagnosis is easy... if you can't find 'em and an alternative diagnosis is not apparent -- you wonder whether you're missing them.

General

Clinical

Symptoms:[1]

  • "B symptoms" - all required:[2] weight loss, night sweats and fever.
  • Infections due to immune dysfunction.

Diagnosis:

  • HL cannot be diagnosed with standard flow cytometry (FC) - but has been diagnosed with specialized FC.[3]

Prognosis:

  • Usually good.
  • Unlike non-Hodgkin lymphomas, it spreads in a predictable pattern; thus, staging plays an important role in determining the therapy.[4]

Hodgkin lymphoma subtypes

Types:[1]

  • Classical HL (CHL) - ~95% of HL.
  • Nodular lymphocyte-predominant HL (NLPHL) - ~5% of HL.

Classic HL

  • Four types of classic HL (see below).

NLPHL

  • AKA lympho-histiocytic variant.
  • Abbreviated NLPHL.
  • Different IHC and morphologic appearance than classic HL.
  • Significant risk for transformation into diffuse large B cell lymphoma (DLBCL); 10-year cumulative transformation rate (to DLBCL) in one study was 12%.[5]

Gross

Location:

  • Almost always arises from a lymph node - classically in the neck.
    • Extranodal Hodgkin lymphoma is (case report) rare.[6]

Microscopic

By definition, HL has Reed-Sternberg cells (RSCs) or Popcorn cells.

Classical HL

Features (classic HL):

  • Reed-Sternberg cell.
    • Large binucleated cell (>= 45 micrometres).[7]
      • May be multinucleated.
      • May have a horseshoe-like shape.
    • Macronucleolus - approximately the size of a RBC (~8 micrometers).
    • Well-defined cell border.
    • Abundant cytoplasm.

Notes:

  • Large mononuclear cells are common (so called "mononuclear RSCs") but not diagnostic.

Images (classic HL)

Subtypes of classic HL

There are four CHL subtypes:[1]

  1. Nodular sclerosis CHL - ~70% of CHL.
    • Mixed cellular background - T cell, plasma cells, eosinophils, neutrophils and histiocytes.
    • Nodular sclerosing fibrosis - thick strands fibrosis.
  2. Mixed cellularity CHL - ~20-25% of CHL.
    • Like nodular sclerosis - but no fibrosis.
    • May be associated with HIV infection.[8]
  3. Lymphocyte-rich CHL - rare.
    • T lymphocytes only (no mix of cells).
  4. Lymphocyte-depleted CHL - rare.
    • May be associated with HIV infection.[8]

Memory device:

  • The subtypes prevalence is in reverse alphabetical order.

Nodular lymphocyte predominant Hodgkin lymphoma

Features (nodular lymphocyte-predominant Hodgkin's lymphoma):

  • Popcorn cell (previously known as Lymphocytic & histiocytic cell (L&H cell)[9]) - variant of RSC:
    • Cells (relatively) small (compared to classic RSCs).
    • Lobulated nucleus - key feature.
    • Small nucleoli.
  • Subtle nodularity at low power (2.5x or 5x objective).

Images (NLPHL)

www:

DDx both CHL & NLPHL

IHC

Abbreviated panel:[11]

  • CD30 Reed-Sternberg cells (RSCs) +ve ~98%
  • CD15 Reed-Sternberg cells +ve ~80%, stains neutrophils.
  • CD45 often negative in RSCs.
  • CD20 may stain RSCs.
  • PAX5 +ve.[12]

Additional - for completeness:

  • CD3 (T lymphocytes)

NLPHL IHC differs from the classical HL:[12]

  • LCA +ve.
  • CD20 +ve.
  • CD10 +ve.
  • Bcl-6 +ve.
  • EMA +ve.
  • CD30 -ve
  • CD15 -ve.

A panel

Antibody NLPHL CHL
CD45 +ve -ve
CD20 +ve -ve
BCL6
MUM1[13] -ve
CD30 -ve +ve (most sensitive).
CD15 -ve +ve
CD21 networks present no networks
CD23 networks present no networks
OCT-2 +ve -ve
PAX5 +ve +ve (proves B cell linage)
CD3 usu. < benign B cell usu. > benign B cell component
CD57 rosettes around malign. cells -
EBER -ve +ve/-ve
EMA +ve/-ve -ve
4 unstained

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Suggestive FNA

Lymph Node, Right Neck, FNA:
	- Large binucleated and multinucleated cells with macronucleoli in 
	  a background of abundant lymphocytes, histiocytes, rare eosinophils. 

Comment:
A cell block is not available for further work-up. The findings raise the possibility of Hodgkin's lymphoma. 

A further biopsy is required for the diagnosis.

See also

References

  1. 1.0 1.1 1.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 567. ISBN 978-0781765275.
  2. URL: http://lymphoma.about.com/od/symptoms/f/bsymptoms.htm. Accessed on: 11 August 2010.
  3. Fromm JR, Thomas A, Wood BL (March 2009). "Flow cytometry can diagnose classical hodgkin lymphoma in lymph nodes with high sensitivity and specificity". Am. J. Clin. Pathol. 131 (3): 322–32. doi:10.1309/AJCPW3UN9DYLDSPB. PMID 19228638.
  4. Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 315. ISBN 978-1416054542.
  5. Biasoli I, Stamatoullas A, Meignin V, et al. (February 2010). "Nodular, lymphocyte-predominant Hodgkin lymphoma: a long-term study and analysis of transformation to diffuse large B-cell lymphoma in a cohort of 164 patients from the Adult Lymphoma Study Group". Cancer 116 (3): 631–9. doi:10.1002/cncr.24819. PMID 20029973.
  6. Vadmal, MS.; LaValle, GP.; DeYoung, BR.; Frankel, WL.; Marsh, WL. (Dec 2000). "Primary localized extranodal hodgkin disease of the transverse colon.". Arch Pathol Lab Med 124 (12): 1824-7. doi:10.1043/0003-9985(2000)1241824:PLEHDO2.0.CO;2. PMID 11100066.
  7. Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 329. ISBN 978-1416054542.
  8. 8.0 8.1 Sissolak G, Sissolak D, Jacobs P (April 2010). "Human immunodeficiency and Hodgkin lymphoma". Transfus. Apher. Sci. 42 (2): 131–9. doi:10.1016/j.transci.2010.01.008. PMID 20138008.
  9. Küppers R, Rajewsky K, Braeuninger A, Hansmann ML (March 1998). "L&H cells in lymphocyte-predominant Hodgkin's disease". N. Engl. J. Med. 338 (11): 763–4; author reply 764–5. doi:10.1056/NEJM199803123381113. PMID 9499174.
  10. Gualco, G.; Natkunam, Y.; Bacchi, CE. (Jan 2012). "The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases.". Mod Pathol. doi:10.1038/modpathol.2011.200. PMID 22222636.
  11. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 568. ISBN 978-0781765275.
  12. 12.0 12.1 Lefkowitch, Jay H. (2006). Anatomic Pathology Board Review (1st ed.). Saunders. pp. 683. ISBN 978-1416025887.
  13. URL: http://www.ncbi.nlm.nih.gov/omim/601900. Accessed on: 10 August 2010.