Lymphoproliferative and myeloproliferative disorders Flashcards
1
Q
Jessner’s epidemiology
A
- middle aged adults
- no gender predilection
2
Q
Jessner’s pathogenesis
A
- Controversial entity: some believe it is a variant of lupus or PMLE, versus pseudolymphoma
- Co-occurrences with: lupus, PMLE, Lyme disease
- Rarely: drug induced: ACEI, glatiramer acetat
3
Q
Jessner’s Clinical
A
- Distribution: head, neck, upper back (think acne)
- Asymptomatic erythematous papules, plaques and nodules. Can have annular plaques with central clearing
- No epidermal change
- Last several weeks to months
- No systemic involvement
- Spontaneous resolution occurs, but can recur
4
Q
Jessner’s Histology
A
- Grenz zone
- No, or very sparse, interface dermatitis
- Superficial and deep peri-vascular lymphocytic infiltrate that may be peri-appendageal
- Mildly increased mucin
- Immunohistochemistry:
- Mixed T-cell infiltrate with a predominance of CD8 lymphocytes
- Mixed with CD123 plasmacytoid denritic cells
- Distribution of the dendritic cells is identical to what is seen in LE tumidus
5
Q
Jessner’s ddx
A
- Infectious: borderline lepromatous leprosy
- Immune: PMLE, lupus tumidus, granuloma faciale
- Infiltrates: pseudolymphoma, REM
- Neoplastic: cutaneous lymphoma
6
Q
Jessner’s treatment
A
- PAGED:
- education: can resolve spontaneously within months to years, without scarring
- TOPICAL
- Topical steroids and calcineurin inhibitors
- Oral systemic:
- 50% improvement with plaquenil
- 76% improvement with thalidomide compared to 16% placebo (crossover study)
- Procedural:
- excision
- resistant to radiation therapy,
- cases of PDL and chemo and PDT
- Caution light as ??lupus/PMLE ddx
- Intra-lesional steroids –> limited success
- Camouflage and cosmetic
7
Q
Pseudolymphoma epidemiology
A
Children and adults
8
Q
Pseudolymphoma pathogenesis
A
- Exaggerated local immunologic reaction to stimulus (often underrecognised)
- Hapten driven immunologic response –> cells damaged by toxic effect of stimuli
9
Q
Pseudolymphoma causative agents
A
- Anything that externally compromises the skin barrier
- Arthropod bites
- Tattoos
- Vaccinations
- Contact allergens
- Contact allergens
- Metal implants
- Infections
- Lyme disease
- Herpes Zoster
- Medications - has a crossover with DRESS drugs. You can have DRESS patients who have atypical lymphocytes in the blood and skin
- Anti-convulstants
- Anti-arrhythmics
- Antibiotics
- Antidepressants
- Anti-histamines
- Antihypertensives
- Anti-psychotics
- Chemotherapy
- Lipid lowering drugs
- NSAIDs
- Allopurinol, dapsone, etc
- Benzos
- Steroid hormones
- Can also be unknown!
10
Q
Pseudolymphoma clinical
A
- Single, firm 1-3 cm erythematous to violaceous plaque or nodule
- Head, neck or upper extremities
- Can be multiple, clustered papules
- Can be large panniculitis like nodules
- Lacks scale
11
Q
B cell Pseudolymphoma histology
A
- Superficial and deep nodular or diffuse infiltrate of lymphocytes
- Admixed histiocytes, and occasional plasma cells and eosinophils
- When florid: germinal centres with prominent tingible body macrophages
12
Q
T cell Pseudolymphoma histology
A
- CD4 T helper lymphocytes are commonly observed within the dermis, admixed with a minority of CD8 cytotoxic/suppressor T cells
- Drug induced - may have an MF-like pattern
- May see: epidermotropism, spongiosis, vacuolar degeneration, papillary dermal oedema, red cell extravasation
- There is no prominent papillary dermal fibrosis (which you see in MF), but T cell clonality may be present
13
Q
B cell Pseudolymphoma immunohistochemistry and clonality analysis
A
Immunohistochemistry
- Distinct B and T cell compartments
- CD35 or CD21 visualize networks of follicular dendritic cells within reactive germinal centres
- Germinal centre cells have a normal CD20, CD10, BCL6, BCL2
- High proliferative rate: MIB-1
Clonality analysis
- Clonality of IgH gene rearrangement or restricted kappa or delta expression is not seen in pseudolymphoma
- Detection of monoclonal expression of immunoglobulin light chains kappa or delta is a key diagnostic feature for primary cutaneous marginal zone B cell lymphoma. Monoclonaltiy is >10:1 ratio
14
Q
How is Pseudolymphoma different to follicle centre B cell lymphoma?
A
- It has a mixed cellular infiltrate such as eosinophils and plasma cells. B cell lymphoma is predominantly lymphocyte
- It has a reactive germinal centre with a mantle zone and tingible body macrophages present
- It has polarization of follicles with light and dark areas. B cell lymphoma has monomorphous appearance of follicles without polarization
- Immunophenotypically it has:
- T and B lymphocytes. B cell has CD20 B lymphocytes
- Bcl-6 cells are restricted to lymph follicles. in B cell lymphoma the Bcl-6 cells are outside of lymph follicles
- Bcl-2 are only on T lymphocytes.
- High proliferation of germinal centres
- Mixed kappa and delta expression (restricted in B cell lymphoma)
15
Q
CD30 Pseudolymphoma histology
A
- persistent arthropod bite reactions, drug reactions, atypical lymphoid infiltrates associated with cutaneous poxvirus infections.
- Has polyclonal arrangement of TCR
16
Q
Pseudolymphoma ddx
A
External: Trauma: arthropod bites, tattoo reaction Infective: Lyme disease, Syphilis Drug: eruptions Internal: Papulosquamous: lichen sclerosus, PLEVA, PPD, contact dermatitis Immune: lupus Infiltrates: pseulymphomatous folliculitis
- Neoplastic:
- Primary cutaneous marginal zone lymphoma
- some argue that these are on the same spectrum
- PCMZL has plasma cells at the periphery of lymphocyte infiltrate
- Demonstration of monotypic palsma cells with either kappa or lambda light chains distinguishes PCMZL
- Primary cutaneous follicle centre lymphoma
- Presence of eosinophils favours pseudolymphoma
- Secondary cutaneous follicular lymphoma
- Primary cutaneous marginal zone lymphoma
Rare: APACHE, IgG4, plasmacytosis, hydroa vaccinforme, Kikuchi Fujimoto
17
Q
What is a tingible body macrophage
A
- Type of macrophage in germinal centres that contains phagocytosed, apoptotic cells which are degrading
- Tingible means: staining
- May play a role in downregulating germinal centre reaction .–> released prostaglandins, and hence a reduced B-cell induction of IL-2
18
Q
APACHE
A
- Acral pseudolymphomatous angiokeratoma of children
- Favours the extremities of children between 2-16 years
- Unilateral grouping of small, red-violet angiomatous papules
- Histologically: dermal infiltrates of lymphocytes, histiocytes and plasma cells with prominently thickened capillaries
19
Q
Kikuchi-Fujimoto: histiocytic necroziting lymphadenitis
A
- Idiopathic systemic inflammatory disease
- Young adult women
- Associations: viral (EBV, CMV), bacterial and autoimmune
- Systemic: fevers, weight loss, GIT symptoms, cervical lymphadenopathy
- Cutaneous: 40% of patients –> acneiform eruptions, urticaria, ulcers, indurated erythematous plaques
- Histo:
- Of skin: dense superficial and deep perivascular lymphohistiocytic infiltrates with nuclear debris (without neutrophils) and interface change
- Histology of nodes: necrosis and histiocytic infiltration of the paracortical areas of involved lymph nodes (without identifiable pathogen)
20
Q
Pseudolymphomatous folliculitis
A
- Solitary facial nodule
- Peri-follicular and peri-adnexal dermal infiltrate
- Infiltrate is mixed, with T or B dominating. There may be cellular atypia and granulomas
- Irregular hyperplasia and distortion of the follicular epithelium
- Blurring of DEJ, CD1a/S100 mononuclear cells surround and infiltrate these distorted follicles
- Needs to be distinguished from CD4 T cell lymphoproliferative disorder (has more pleomorphism, and has PD1 expresion)
21
Q
Cutaneous and systemic plasmacytosis
A
- Benign condition
- Aetiology unknown - ?reactive dysfunction of plasma cells triggered by various stimuli
- Similar to Castlemans: elevated IL-6 in blood and tissue, but no HHV-8 infection
- Seen in Asians (particularly Japanese)
- Multiple reddish-brown, infiltrated maculopapules and plaues
- Distribution: commonly the trunk
- Systemic: lymphadenopathy, hepatosplenomegaly, interstitial penumonia, mesangial proliferative GN
- +/- anaemia, B symptoms, hypergammaglobulinaemia
- Histology: dense superficial and deep peri-vascualr infiltrates of mature polyclonal plasma cells
- To demonstrate polyclonality: kappa and lambda immunohistochemistry or FISH
22
Q
Pseudolymphoma treatment
A
- Benign –> treat conservatively
- May resolve without scarring
- Persistent lesions:
- Topical steroids
- Oral systemic: thalidomide for recalcitrant, or treat Lyme disease
- Procedural:
- simple excision
- cryosurgery
- laser ablation
- radiation therapy
- Biopsy –> can stimulate regression
- Intra-lesional steroids
- Camouflage
- Monitor
23
Q
Extramedullary Haematopoiesis epidemiology
A
- Neonates
- Can occur in adults with myelofibrosis and less often myelodysplasia or after a splenectomy
24
Q
Extramedullary Haematopoiesis pathogenesis
A
- Reflects bone marrow dysfunction
- Occurs during early embryogenesis and usually stops prior to birth
- After that, it can occur as a secondary phenomenon when the bone marrow function is altered
25
Extramedullary Haematopoiesis clinical
- Erythematous to violaceous papules and nodules, that may ulcerate
- Blueberry muffin baby: widely disseminated. Can be seen in associated with congenital viral infections and prenatal anaemias
- Dermal haematopoiesis is most commonly seen with:
- Rubella
- CMV
- Associated cutaneous neoplasms: PPHIS
- Pilomatricoma
- Pyogenic granuloma
- Haemangioma
- Sebaceous naevus
26
Blueberry muffin baby syndrome Ddx
- External
- Infections:
- Pre-natal
- TORCH
- Coxsackie
- Parvovirus
- Internal
- Neoplastic
- Myelodysplasias
- Congenital Leukaemia
- Congenital alveolar rhabdomyosarcoma
- Congenital LCH
- Neonatal neuroblastoma
- Congenital leukaemia cutis
- Vascular
- Haemangiomatosis
- Glomuvenous
- Multifocal lymphangioendothleiomatosis
- Disseminated extramedullary haematopoiesis
- Metabolic
- Anaemias: pre-natal, haemolytic, twin-twin transfusion
- Haemorrhage
27
Extramedullary Haematopoiesis histology
- Dermal infiltrate of immature red and white blood cell precursors and megakaryocytes, centred around vessels of the superficial vascular plexus
- Can extend into the deeper reticular dermis and exhibit a diffuse pattern
- All three haematopoietiic cell lines are present in varying ratios: erythrocytes, leukocytes, megakaryocytes
28
Extramedullary Haematopoiesis ddx
- limited histologically
- Distinguishing it from congenital leukaemia cutis and neonatal neuroblastoma is important --> leukaemia cutis has just leukocytes and not the others
- Patients with overwhelming marrow involvement from leukaemia may have both EMH and leukaemic involvement of the skin which may make things confusion
29
Extramedullary haematopoiesis treatment
- treat the underlying BM dysfunction
| - if viral, can have spontaneous resolution
30
Leukaemia cutis epidemiology
- ALL --> kids
- AML and CML --> adults
- CLL and hairy cell leukaemia --> elderly
- When see with CML --> can be blast transformation
- When see with myelodysplatic syndrome --> transformation to AML
31
Leukaemia cutis pathogenesis
- CML:
- Philadelphia chromosome: translocation between chromosomes 9 and 22 --> results in fusion of BCR and ABL --> activation of tyrosine kinase activity of the fusion oncoprotein. This is targeted by imatinib
- Promyelocytic leukaemia --> T(15;17) translocation which leads to abnormal expression of a fusion retinoic acid receptor that is the target of all-trans retinoic acid therapy
- Non-random chromosomal abnormalities exists in ALL and AML that can influence prognosis
- Mutations can be favourable and unfavourable
- CLL:
- 13q deletion is good
- Trisomy 12 is unsure
- Deletions of 6q, 11q and 17 p are bad
32
Leukaemia cutis: non-specific reactive skin lesions and what are they most commonly seen with
- Neutrophilic dermatoses
- Sweets - AML>CML, hairy cell leukaemia
- Pyoderma gangrenosum - AML, CML, hairy cell leukaemia >ALL, CLL
- Neutrophilic eccrine hidradenitis - AML the most
- Reactive erythemas
- Exaggerated arthropod reactions and erythroderma - CLL
- Facial erythema and oedema - T cell prolymphocytic leukameia
- Vasculitis
- PAN - hairy cell leukamia, CMML
- Vasculitis - hairy cell, CLL > AMML, AML
- EED - hairy cell leukaemia, CLL
- Panniculitis
- EN - AML, CML, CMML
- Other panniculitides - hairy cell, AML, CMML
- Other
- CLL:
- urticaria
- erythema multiforme
- paraneoplastic pemphigus
- adult onset eczema (recalcitrant)
- Acral ischaemia with lividity - CML
- Granulomatous reactions: MDS
- Pernio: CMML
33
Leukaemia cutis specific infiltrates
- Firm papules and nodules that often become haemorrhagic
- Thrombocytopaenia usually plays a role in the bleeding
- Rarely bullous and ulcerative
- Distribution: head, neck, trunk, but anywhere, and also arise at sites of trauma or scars
- Myelogenous leukaemias may present with dermal nodules: chloromas, granulocytic sarcomas, extramedullar myeloid tumours
- Granulocytic sarcomas may precede the development of systemic leukaemia
- Gingival hyerplasia, secondary to leukaemic infiltrates, favours acute monocytic or acute myelomonocytic leukaemia
- Very rarely, skin involvement precedes the leukaemia diagnosis
- Rarely, leukaemia cutis predates BM involvement by months or even years
34
Chloroma
- Arise in myeloid leukaemia or granulocytic sarcoma
- Can present as solitary or numerous nodules
- Appear blue-green --> due to myeloperoxidase granules in the malignant leukocytes
- May precede development of systemic leukaemia by several months
35
ALL overview
- <6 years of age
- Head and neck
- Aggressive
- Predominantly B-cell ALL
- Rarely get cutaneous involvement
- Large, non-ulcerated tumours, nonspecific papulonodules
36
CLL overview
- 70s or older
- Generalised, may appear at sites of infection
- Indolent course
- B cell type
- Relatively common cutaneous involvement
- Non-specific papulonodules
37
AML overview
- 70s
- Generalised distribution, aggressive
- Most commonly AMML, AMoL
- Cutaneous invovlement uncommon, nonspecific papulonodules
- Early presentation
38
CML overview
- most commonly after 50 years of age
- Most commonly CMML
- Cutaneous involvement rare, but associated with blast transformation
- Nonspecific papulonodules
39
AML histology
- neoplastic proliferation of immature myeloid cells
- Cytoplasmic granules
- Stain: chloroacetate esterase --> helpful in detecting myeloid differentiation, but when very early may not have granules
- Most common forms in skin are de novo
- Stains: myeloperoxidase, CD13, CD33 and CD68
40
ALL histology
- Clonal expansion of immature lymphocytes, more commonly B cell
- Rarely, cytoplasmic granularity
- Stain: terminal deoxytransferase
41
CML histology
- Demonstrates a range of myeloid precursors - promyelocytes, metamyelocytes, bands, mature neutrophils
- Cutaneous is rare, but associated with blast transformation
42
CLL histology
- Dense infiltrate of uniform appearing, small, round lymphocytes of B-cell lineage
- cells appear mature, but monomorphous nature and paucity of other cell types suggest leukaemic infiltrate
- peri-adnexal and vascular
- Stain: CD5, CD20, CD43
43
Leukaemia cutis ddx
- Lymphoma cutis
- Infectious emboli
- Vasculitis
- Drug eruptions
- Myeloid leukaemias
- EMH
- Sweets --> composed mostly of immature cells of meyloid lineage so can be misinterpreted
- Small vessel vasculitis
- Neutrophilic dermatoses
44
Cutaneous Hodgkin Lymphoma epi
- utaneous involvement is rare --> 0.5% with HL develop cutaneous involvement
- Occurs in advanced disease
- Sign of poor prognosis
- HL peaks in early adulthood, with second, smaller peak in sixth decade
- MF and LyP have higher rates of occurrence now
45
Cutaneous Hodgkin Lymphoma pathogenesis
- 30-40% with classic HL have EBV DNA within the Reed-Sternberg cells
- Role of EBV is not known
- Reed-Sternberg cells represent the malignant population, and are usually derived from B-cell lymphocytes
46
Cutaneous Hodgkin Lymphoma clinical
- Classic HL includes:
- Nodular sclerosing (most common)
- Mixed cellularity
- Lymphocyte-depleted
- Non-classic: more indolent --> nodular lymphocyte-predominant HL
- Cutaneous involvement:
- Multiple papulonodules or plaques
- Trunk most common
- Lesions often distal to affected lymph nodes
- Extra-cutaneous:
- Lymphadenopathy
- Splenomegaly
- Constitutional symptoms
- 20-50% paraneoplastic cutaneous disorders: primary pruritis, acquired ichthyosis, persistent dermatitis, hyperpigmentation, EN
47
Cutaneous Hodgkin Lymphoma histology
- Nodular or diffuse dermal infiltrate: lymphocytes, eosinophils, plasma cells
- No epidermotropism
- Reed-Sternberg cells and lacunar cells may be present
- Lacunar cells: large cells with abundant eosinophilic cytoplasm. Retraction fo cytoplasm from adjacent cells during fixation results in a giant cell residing within a clear space
- Dermal sclerosis may be seen --> extension into S/C fat is common
48
Cutaneous Hodgkin Lymphoma immunohistochemistry
- R-S cells express: CD30 (Ki-1), fascin, CD15, PAX-5, MUM-1, negative for CD45RB and CD20.
- B/G of lymphocytes predominantly T cells
- In lymphocyte predominant HL, the malignant cells are positive for CD20
49
Cutaneous Hodgkin Lymphoma ddx - important ones
- cALCL --> stains positive for CD30. Negative for CD15 and PAX-5
- LyP --> don't have systemic involvement. Negative for CD15 and PAX-5
50
Cutaneous Hodgkin Lymphoma treatment
- Stage 4: cure rate of 60-70% following combination chemotherapy
- Recurrent disease: salvage chemo, autologious HSCT, anti-CD30 (brentuximab) and anti-PD1 antibodies
51
Blastic Plasmacytoid Dendritic Cell Neoplasm epidemiology
- extremely rare
- is a distinct entity
- grouped with AML-related precursor neoplasms
- Disease of adults, >50 years
- M>F 3:1
52
Blastic Plasmacytoid Dendritic Cell Neoplasm pathogenesis
- Malignant proliferation of plasmacytoid dendritic cells
- These cells produce large amounts of type 1 inferferons: interferon alpha in particular, and TLR --> making them critical in defense against viruses and other pathogens
53
Blastic Plasmacytoid Dendritic Cell Neoplasm clinical
- Solitary or multiple
- Non-tender, occasionally pruritic
- Erythematous-violaceous papules, nodules, plaques that can look like bruises
- Ulceration is common
- Skin is often the presenting site of involvement
- Evaluation: regional lymph nodes, peripheral blood, bone marrow involvement
- Those with initial negative staging eventually develop blood invovlement
- Constitutional: fevers, chills, night sweats, weight loss --> uncommon but do occur as the disease progresses
- Isolated reports of developing AML
54
Blastic Plasmacytoid Dendritic Cell Neoplasm histology
- Grenz zone
- Diffuse dermal infiltrate of monotonous, atypical, medium-sized mononuclear cells
- Cells: fine blastic chromatin, indistinct nucleoli, scant agranular eosinophilic cytoplasm
- Intra-tumoral haemorrhage is common
- Early lesions: infiltrate may be peri-vascular and peri-adnexal
55
Blastic Plasmacytoid Dendritic Cell Neoplasm immunohistochemistry
- Positive: CD4, CD56, CD123 (presence of plasmacytoid dendritic cells - PDC, its easy as 123), TCL1, BDCA2, CD303
- Negative: myeloperoxidase and EBV
- Tumour cells have germline configurations of Ig(H&L) and TCR (i.e. lack rearrangement)
56
Blastic Plasmacytoid Dendritic Cell Neoplasm genetics
- CDKN1B - 12p13
- RB1 - 13q13
- CDKN2A - 9p21
- These genes can also be altered in NHL, ALL and hairy cell leukaemia
57
Blastic Plasmacytoid Dendritic Cell Neoplasm ddx
- Neoplastic:
- AML
- Other cutaneous lymphomas
- BPDCN lacks IgH and TCR gene rearrangement, and has a really characteristic immunohistochemical profile
- Expression of TCL1, BDCA2 and CD2AP diagnoses it
58
Blastic Plasmacytoid Dendritic Cell Neoplasm Rx
- Multidrug chemotherapy --> response rates are high (up to 80%) but relapses rare common --> 1 year median overall survival
- Allogeneic HSCT
- Needs aggressive treatment
- Aberrant activation of NF-kB pathway may represent a therapeutic target
59
Angioimmunoblastic T-cell lymphoma - AITCL epidemiology
- Very rare
- Midle-aged and elderly
- M=F
60
Angioimmunoblastic T-cell lymphoma - AITCL pathogenesis
- Considered a mature T-cell neoplasm whose cells of origin are follicular T helper cells
- Frequent progression to aggressive lymphoma, has clonal rearrangement of TCR
61
Angioimmunoblastic T-cell lymphoma - AITCL clinical
- Usually preset at advanced stage: fever, weight loss, night sweats, generalized lymphadenopathy, hepatosplenomegaly
- 1/2 cutaneous:
- Widespread morbilliform that looks like a viral exanthem
- Petechiae
- Urticaria
- Purpura
- Papulonodules
- Rarely erythroderma
- Generalised pruritis
- Aggressive: median survival < 3 years
62
Angioimmunoblastic T-cell lymphoma - AITCL histology patterns
1. Superficial perivascular infiltrate of eosinophils and lymphocytes (most common) that lack atypia
2. Sparse perivascular infiltrate with atypical lymphocytes
3. Denser superficial and deep infiltrate of pleomorphic lymphocytes
4. Vasculitis (+/- atypical lymphocytes)
5. Necrotizing granulomas
- Skin may have increased numbers of venules with a prominent endothelial lining
63
Angioimmunoblastic T-cell lymphoma - AITCL immunohistochemistry and gene mutation
- Corresponds to follicular T helper cells: CD3+, CD4+, CD8-, CD10+, PD-1+, ICOS+, Bcl-6+, CXCL13+
- EBV negative
- Molecular analysis: monoclonal TCR rearrangement and polyclonal immunoglobulin pattern
- Genee mutation: TET2 --> protein product is tet methylcytosine dioxygenase 2 --> involved in DNA methylation, and is implicated in T cell lymphoma
64
Angioimmunoblastic T-cell lymphoma - AITCL ddx
- drug eruption
- viral exanthem
- Histo changes are subtle
65
Angioimmunoblastic T-cell lymphoma - AITCL rx
- Majority present with Stage 3-4
- Prospective RCT: no observable differences among varying regimens, even with HSCT
- 46% response with induction, but 5 year survival is 33% and 7 year is 29%
66
Lymphomatoid Granulomatosis epidemiology
- Rare
- 50s-60s
- M>F 2:1
- Can affect children, in immunodeficiency syndromes
67
Lymphomatoid Granulomatosis pathogenesis
- EBV (+/- immunosuppression)
| - Immunosuppressive factors: renal transplant, Wiscott Aldrich, HIV, X-linked lymphoproliferative syndrome
68
Lymphomatoid Granulomatosis clinical
- Pulmonary: cough, dyspnoea, hcest pain
- Constitutional: fever, weight loss, malaise, arthralgias, myalgias
- Cutaneous
- 25-50% of aptients
- Nodules or ulcerated plaques
- Maculopapular exanthem
- Can affect kidney, brain, GIT, rarely LN and spleen
69
Lymphomatoid Granulomatosis histology grades
Three Grades:
1. Grade 1: polymorphous lymphoid infiltrate without cytologic atypia, a few large cells, a few EBV positive cells (EBER-1)
2. Grade 2: polymorphous inflammatory background with scattered large cells and numerous EBV positive cells (EBER-1)
3. Grade 3: Large sheets of B cells and numerous EBER-1 cells over an inflammatory background
- amount of necrosis increases with grade and angiocentricity/angiodestruction
- Granulomatous change
70
Lymphomatoid Granulomatosis stains
- CD20
- CD79a
- Some cases: monoclonal rearrangement of Jh gene
71
Lymphomatoid Granulomatosis ddx
- Neoplastic
- Cutaneous lymphoma
- Infectious
- Granulomatous
- Immune
- Medium vessel vasculitis
- Infiltrates
- Pyoderma gangrenosum
- Granulomatous - Wegeners, sarcoid
72
Lymphomatoid Granulomatosis rx
- Aggressive course - 5 year mortality ranges from 60-90%
- Higher grade disease - treatment commonly involves multidrug chemotherapy and/or rituximab
- Grade 1 disease - interferon alpha
- Reversal of exogenous immunosuppression will also lead to improvement
