Other lymphoproliferative and myeloproliferative diseases Flashcards
What cancer is most commonly associated with leukemia cutis?
AML
Usually, there is proceeding marrow and peripheral blood involvement but you can have aleukemic leukemia cutis as well
What is the clinical appearance of leukemia cutis?
Violaceous papules and nodules at any location. Note these can have a green appearance
What subforms of AML are most associated with leukemia cutis?
Myelomonocytic and monocytic
What is the histology of leukemia cutis?
Grenz zone
- diffuse dermal infiltrate of myeloid blasts
- These cells are monotonous and w/ high nuclear to cytoplasm ratio and fine chromatin.
- Can look like they are in sheets, nodules, perivascularly or infiltrative cords (Indian filing)
What is the most common staining for leukemia cutis?
MPO+, CD117 (c-kit)+, CD13+, CD33+, and CD34+
What is the name of the green appearing nodules in the setting of AML?
Chloroma
What is the cause of the green appearance in a chloroma?
Myeloperoxidase activity
What is the differential diagnosis for red indurated plaques involving the face?
Lupus erythematosus, PMLE, Lymphoma Cutis, Lymphocytoma cutis, Jessner’s lymphocytic infiltrate
What is the clinical appearance of Jessner’s lymphocytic infiltrate?
1 or several asymptomatic erythematous papules,
plaques, and less commonly, nodules, with the absence of secondary changes like scale on head, neck, and upper back
May appear as annular plaque with central clearing if individual lesion
No associated systemic manifestations
What is the treatment of Jessner’s Lymphocytic Infiltrate?
Resolves spontaneously within months to years, without scarring or sequelae
50% pts may improve with hydroxychloroquine
Thalidomide, pulsed dye (595 nm) laser, chemotherapy as other options
What is the histology of Jessner’s lymphocytic infiltrate?
Superficial and deep perivascular and periadnexal lymphocytic infiltrate
interface dermatitis is absent
predominance of CD8+ lymphocytes
What is the cause of cutaneous lymphoid hyperplasia?
Reflects exaggerated local immunologic reaction to a stimulus, often unrecognized
Inciting agents: arthropod bites, metal implants, tattoos, vaccinations and medications as well as several infections and reactive disorders
What is the clinical appearance of cutaneous lymphoid hyperplasia?
Firm, erythematous to violaceous papules, plaques or nodules located on head, neck or upper extremities
Often solitary but may be multiple and grouped
Vast majority lack surface changes
Most often in adults; slightly more common women
What can cutaneous lymphoid hyperplasia also be called?
Pseudolymphoma (can resemble lymphoma or have a more benign appearance)
What is the treatment for cutaneous lymphoid hyperplasia?
Treat conservatively, spontaneous resolution may occur without scarring; topical or IL steroids for persistent lesions; simple excision, cryosurgery, laser ablation, and radiation therapy; thalidomide for recalcitrant cases
What type of leukemia is most often associated with neutrophilic disorders (Sweet’s, pyderma gangrenosum, and neutrophilic eccrine hidradenitis?
AML
In what leukemias can you see exaggerated arthropod reactions or erythroderma?
CLL
What is the most common leukemia associated with polyarteritis nodosa, vasculitis (small vessel/leukocytoclastic), erythema elevatum diutinum?
Hairy cell leukemia
What leukemias are most commonly associated w/ erythema nodosum?
AML, CML, CMML
What are the leukemias most associated with other panniculitides aside from erythema nodosum?
Hairy cell leukemia, AML, CMML
What leukemia is most commonly associated with erythema multiforme?
CLL
What leukemias are most commonly associated with urticarial?
CLL, hairy cell leukemia
What is the immunophenotype ALL?
Most commonly TDT+, CD5+, CD19+, CD20+, CD10+ B-cell)
Can rarely be T-cell related as well (TDT+, Cd1a+, CD2+, CD3c+, CD5+, CD7+)
What is the immunophenotype of CLL?
CD5+, CD20+, CD43+ (B-cell types)
What is the most common immunophenotype of AML?
AMML most common (M4 and AMol (M5) =
CD13+, CD33+, CD68+, MPO+
What stain can be helpful in diagnosing leukemia cutis?
Leder stain (can identify the atypical cells as myeloid)
What is the prognostic significance of leukemia cutis?
Poor prognostic finding. 90% of patients have extramedullary involvement and 40% have meningeal infiltration
In what setting does cutaneous Hodgkin’s occur in?
Advanced systemic dz and is a marker of poor prognosis
What is the most specific stain for Hodgkin lymphoma?
Pax-5
What is the clinical appearance of cutaneous Hodgkin lymphoma?
Isolated cutaneous HD presents as 1 or several papules, plaques, or ulcerated nodules with no site predilection
MC presentation is multiple, disseminated papulonodules and plaques appearing rapidly in pt with known, advanced disease
Often distal to affected lymph nodes
Trunk most common site
20-50% pts can have paraneoplastic cutaneous disorders like primary pruritus, acquired ichthyosis, persistent dermatitis, hyperpigmentation, and erythema nodosum
What are the other systemic cutaneous findings can occur in Hodkin’s lymphoma?
20-50% pts can have paraneoplastic cutaneous disorders like primary pruritus, acquired ichthyosis, persistent dermatitis, hyperpigmentation, and erythema nodosum
What is the histology of Hodgkin lymphoma?
Dermal infiltrate consistent of lymphocytes with occasional eosinophils and plasma cells present
Reed-Sternberg cells and lacunar cells (large cells with abundant eosinophilic cytoplasm) may be present depending on subtype
What is the clinical appearance of blastic plasmacytoid dendritic cell neoplasm?
Adults usually >50 y/o
-Male-to-female ratio 3:1
solitary or multiple, non-tender, variably pruritic, erythematous to violaceous papules, plaques or nodules
Skin is presenting site of involvement in 50% pts
May have subclinical regional lymph node, peripheral blood and bone marrow involvement
What is the pathology of blastic plasmacytoid dendritic cell neoplasm?
Diffuse dermal infiltrate of monotonous, atypical, medium-sized mononuclear cells
Fine blastic chromatin, indistinct nucleoli and scant granular eosinophilic cytoplasm
Numerous mitoses
Grenz zone
Infiltrate commonly extends to subcutis
Immunohistochemical studies: CD4+, CD56+, CD123+, TCL1+, BDCA2+, MPO-, TCL1+ 90% cases
What is the immunophenotype of blastic plasmacytoid dendritic cell neoplasm?
CD4+, CD56+, CD123+, TCL1+, BDCA2+, MPO-, TCL1+ 90% cases
What are the 5 histologic patterns of angioimmunoblastic T-cell lymphoma?
- Superficial perivascular infiltrate composed of eos and lymphocytes that lack atypia (MC)
- Sparse perivascular infiltrate with atypical lymphocytes
- Dense superficial and deep infiltrate of pleomorphic lymphocytes
- Vasculitis with or without atypical lymphocytes
- Necrotizing granulomas
What is the clinical presentation of angioimmunoblastic T-cell lymphoma?
- constitutional sx’s: fever, sweats, weight loss)
- Variable morphology: morbilliform erythema, urticaria, and rarely erythroderma
- Can have generalized LAD and hepatosplenomegaly
What infection is lymphomatoid granulomatosis related to?
EBV infection sometimes w/ immunosuppression (think renal transplant, Wiskott-Aldrich, HIV, x-linked lymphoproliferative syndrome
What is the epidemiology of lymphomatoid granulomatosis?
Adults, 5-6th decade, male to female ratio 2:1
What are the most prominent cell seen on histology in lymphomatoid granulomatosis?
T-cells! Even though it is a b-cell process there is a reactive process that is more prominent than the underlying dz
What are the clinical features of lymphomatoid granulomatosis
Sxs related to pulmonary involvement – cough, dyspnea, and chest pain (common presenting)
Constitutional sxs like fever, weight loss, malaise, arthalgias and myalgias
Cutaneous lesions in 25-50% pts – nodules or ulcerated plaques
May affect kidney, brain, and GI
Lymph node and spleen rarely involved
What is the histopathology of lymphomatoid granulomatosis?
Nodular lymphoid infiltrates composed of lymphocytes, plasma cells and large atypical mononuclear cells with convoluted nuclei and prominent nucleoli
Often surround and invade arteries and veins
Granulomatosis is distinct prominent feature – necrosis within lymphoid aggregates in absence of granuloma formation
+CD20, +CD79a, +EBV DNA
Immunophenotype in lymphomatoid granulomatosis?
+CD20, +CD79a, +EBV DNA
What is the treatment of lymphomatoid granulomatosis?
Aggressive; 60-90% 5-year mortality; multidrug chemo and/or rituximab; IFN-alpha for milder cases; reversal of exogenous immunosuppression may lead to clinical improvement
