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Pathology - Primary myelofibrosis
A myeloproliferative neoplasm distinguished by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, accompanied by reactive deposition of fibrous connective tissue and extramedullary hematopoiesis.
Epidemiology • Estimated yearly incidence ranges from 0.5 to 1.5 per 100,000 individuals. • Predominantly affects people aged 60 to 70, with no discernible gender preference. Aetiology • Predominantly unknown in the majority of instances. Genetics • No specific genetic anomaly has been identified.
Presentation: Abdominal discomfort resulting from significant splenomegaly. • Nocturnal hyperhidrosis, pyrexia, and weight reduction.
Peripheral blood • Increased platelets and/or white cell count. • Decreased hemoglobin. The blood film exhibits leukoerythroblastosis characterized by teardrop-shaped red blood cells. Bone marrow • Megakaryocytes exhibit significant abnormalities characterized by extensive clustering and pronounced cytological atypia. • Progression is accompanied by an increase in marrow fibrosis.
Prognosis • Survival is contingent upon the degree of marrow fibrosis at the time of diagnosis. Patients exhibiting significant fibrosis have median survival durations of 3 to 7 years. The primary causes of mortality are bone marrow failure, thromboemboli, and the onset of acute myeloid leukemia (AML).
A myeloproliferative neoplasm distinguished by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, accompanied by reactive deposition of fibrous connective tissue and extramedullary hematopoiesis.
Epidemiology • Estimated yearly incidence ranges from 0.5 to 1.5 per 100,000 individuals. • Predominantly affects people aged 60 to 70, with no discernible gender preference. Aetiology • Predominantly unknown in the majority of instances. Genetics • No specific genetic anomaly has been identified.
Presentation: Abdominal discomfort resulting from significant splenomegaly. • Nocturnal hyperhidrosis, pyrexia, and weight reduction.
Peripheral blood • Increased platelets and/or white cell count. • Decreased hemoglobin. The blood film exhibits leukoerythroblastosis characterized by teardrop-shaped red blood cells. Bone marrow • Megakaryocytes exhibit significant abnormalities characterized by extensive clustering and pronounced cytological atypia. • Progression is accompanied by an increase in marrow fibrosis.
Prognosis • Survival is contingent upon the degree of marrow fibrosis at the time of diagnosis. Patients exhibiting significant fibrosis have median survival durations of 3 to 7 years. The primary causes of mortality are bone marrow failure, thromboemboli, and the onset of acute myeloid leukemia (AML).
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Pathology - Myelodysplastic syndromes
Definition: A collection of hematopoietic neoplasms distinguished by dysplasia in one or more myeloid cell lineages, accompanied by inefficient myelopoiesis, cytopenias, and an elevated risk of acute myeloid leukemia (AML) development.
Epidemiology • Estimated yearly incidence ranges from 3 to 5 per 100,000 individuals. • Predominantly affects older adults, with a median age of 70 years.
Aetiology • Predominantly unknown in the majority of instances. Genetics • Several recurrent chromosomal abnormalities have been identified in myelodysplastic syndromes (MDS). Cytogenetic and molecular analyses are essential for establishing clonality and assessing prognosis.
Presentation • Refractory anaemia is the predominant manifestation. • Neutropenia and thrombocytopenia occur with lesser frequency. 2 Hepatosplenomegaly is rare in myelodysplastic syndromes. Peripheral blood • Cytopenias affecting one or more myeloid lineages. • Blood films may reveal macrocytes and atypical neutrophils with poorly developed nuclear segmentation and hypogranular cytoplasm. Bone Marrow • Morphological indicators of myelodysplasia may be observed in one or more myeloid lineages within the bone marrow. • Dyserythropoiesis is defined by nuclear budding, internuclear bridging, karyorrhexis, multinuclearity, nuclear hypolobulation, megaloblastic alterations, ring sideroblasts, and cytoplasmic vacuolization. • Dysgranulopoiesis is characterized by reduced size, nuclear hypolobation, irregular hypersegmentation, and cytoplasmic hypogranularity. • Dysmegakaryocytopoiesis is marked by reduced size, nuclear hypolobation, or multinucleation.
Prognosis: Survival is contingent upon various criteria, including morphological subtype, karyotype, degree of cytopenia, and age. • Low-risk variants of MDS exhibit a protracted natural history and demonstrate a minimal incidence of progression to AML. • High-risk variants are more aggressive, with numerous individuals rapidly succumbing to bone marrow loss or AML.
Definition: A collection of hematopoietic neoplasms distinguished by dysplasia in one or more myeloid cell lineages, accompanied by inefficient myelopoiesis, cytopenias, and an elevated risk of acute myeloid leukemia (AML) development.
Epidemiology • Estimated yearly incidence ranges from 3 to 5 per 100,000 individuals. • Predominantly affects older adults, with a median age of 70 years.
Aetiology • Predominantly unknown in the majority of instances. Genetics • Several recurrent chromosomal abnormalities have been identified in myelodysplastic syndromes (MDS). Cytogenetic and molecular analyses are essential for establishing clonality and assessing prognosis.
Presentation • Refractory anaemia is the predominant manifestation. • Neutropenia and thrombocytopenia occur with lesser frequency. 2 Hepatosplenomegaly is rare in myelodysplastic syndromes. Peripheral blood • Cytopenias affecting one or more myeloid lineages. • Blood films may reveal macrocytes and atypical neutrophils with poorly developed nuclear segmentation and hypogranular cytoplasm. Bone Marrow • Morphological indicators of myelodysplasia may be observed in one or more myeloid lineages within the bone marrow. • Dyserythropoiesis is defined by nuclear budding, internuclear bridging, karyorrhexis, multinuclearity, nuclear hypolobulation, megaloblastic alterations, ring sideroblasts, and cytoplasmic vacuolization. • Dysgranulopoiesis is characterized by reduced size, nuclear hypolobation, irregular hypersegmentation, and cytoplasmic hypogranularity. • Dysmegakaryocytopoiesis is marked by reduced size, nuclear hypolobation, or multinucleation.
Prognosis: Survival is contingent upon various criteria, including morphological subtype, karyotype, degree of cytopenia, and age. • Low-risk variants of MDS exhibit a protracted natural history and demonstrate a minimal incidence of progression to AML. • High-risk variants are more aggressive, with numerous individuals rapidly succumbing to bone marrow loss or AML.
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Pathology - Extranodal marginal zone lymphoma
Definition: An extranodal mature B-cell neoplasm primarily consisting of tiny neoplastic marginal zone cells.
Epidemiology • Constitutes 7–8% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults with a mean age of 60. Locations of engagement • The gastrointestinal system constitutes 50% of all instances, with the stomach as the predominant site. • Additional locations encompass the lung, salivary gland, skin, thyroid, and breast.
Etiology • Gastric instances are generally linked to Helicobacter pylori. • Additional implicated species comprise Campylobacter jejuni (jejunum) and Borrelia burgdorferi (skin). • Autoimmune illnesses are also linked, such as Hashimoto’s thyroiditis (thyroid) and Sjögren’s syndrome (salivary gland). Pathogenesis • The majority of cases are preceded by a chronic inflammatory condition that results in the accumulation of extranodal lymphoid tissue. • Extended stimulation of lymphoid proliferation ultimately culminates in the transition into a neoplastic process.
Presentation • Symptoms associated with a mass at the affected location.
Histopathology • Affected tissues comprise a diverse assemblage of small neoplastic B-cells that encircle and may infiltrate the underlying reactive lymphoid follicles. • The cellular composition includes marginal zone cells, monocytoid-like cells, small lymphocytes, and dispersed immunoblasts and centroblast-like cells. • In epithelial-lined tissues, the neoplastic lymphoid cells generally infiltrate and obliterate the epithelium, resulting in lymphoepithelial lesions. Immunophenotype: B-cell markers PAX5, CD20, and CD79a are expressed positively. CD5, CD10, CD23, and cyclin D1 are all absent.
Prognosis: Exhibits indolent behavior with extended periods of disease-free remission post-treatment.
Definition: An extranodal mature B-cell neoplasm primarily consisting of tiny neoplastic marginal zone cells.
Epidemiology • Constitutes 7–8% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults with a mean age of 60. Locations of engagement • The gastrointestinal system constitutes 50% of all instances, with the stomach as the predominant site. • Additional locations encompass the lung, salivary gland, skin, thyroid, and breast.
Etiology • Gastric instances are generally linked to Helicobacter pylori. • Additional implicated species comprise Campylobacter jejuni (jejunum) and Borrelia burgdorferi (skin). • Autoimmune illnesses are also linked, such as Hashimoto’s thyroiditis (thyroid) and Sjögren’s syndrome (salivary gland). Pathogenesis • The majority of cases are preceded by a chronic inflammatory condition that results in the accumulation of extranodal lymphoid tissue. • Extended stimulation of lymphoid proliferation ultimately culminates in the transition into a neoplastic process.
Presentation • Symptoms associated with a mass at the affected location.
Histopathology • Affected tissues comprise a diverse assemblage of small neoplastic B-cells that encircle and may infiltrate the underlying reactive lymphoid follicles. • The cellular composition includes marginal zone cells, monocytoid-like cells, small lymphocytes, and dispersed immunoblasts and centroblast-like cells. • In epithelial-lined tissues, the neoplastic lymphoid cells generally infiltrate and obliterate the epithelium, resulting in lymphoepithelial lesions. Immunophenotype: B-cell markers PAX5, CD20, and CD79a are expressed positively. CD5, CD10, CD23, and cyclin D1 are all absent.
Prognosis: Exhibits indolent behavior with extended periods of disease-free remission post-treatment.
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Pathology - Follicular lymphoma
Definition: A mature B-cell neoplasm consisting of germinal center cells (centrocytes and centroblasts).
Epidemiology • Represents approximately 20% of all non-Hodgkin lymphomas. • Primarily impacts individuals aged 50 to 60.
Aetiology: Unknown. Genetics: 90% of cases exhibit a distinctive t(14;18) translocation, leading to the fusion of the BCL2 gene with the IGH locus. Deregulated synthesis of the anti-apoptotic Bcl-2 protein leads to clonal proliferation.
Presentation: Extensive lymphadenopathy and splenomegaly. • Patients are often asymptomatic.
Histopathology Nodal architecture is supplanted by contiguous neoplastic follicles. Neoplastic follicles are devoid of mantle zones and consist of haphazardly arranged neoplastic centroblasts and centrocytes. Tangible body macrophages are typically lacking. • Neoplastic cells typically exhibit interfollicular dissemination. Bone marrow involvement is defined by paratrabecular clusters of malignant centrocytes and centroblasts. Immunophenotype PAX5, CD20, and CD79a, which are B-cell markers, exhibit positivity. Bcl-2, Bcl-6, and CD10 are furthermore positive. • CD5, CD23, and cyclin D1 are absent.
Prognosis is contingent upon the severity of the disease and the grade of the tumor. Approximately 25% progression into a high-grade lymphoma, typically diffuse large B-cell lymphoma, correlating with a swift clinical deterioration and mortality.
Definition: A mature B-cell neoplasm consisting of germinal center cells (centrocytes and centroblasts).
Epidemiology • Represents approximately 20% of all non-Hodgkin lymphomas. • Primarily impacts individuals aged 50 to 60.
Aetiology: Unknown. Genetics: 90% of cases exhibit a distinctive t(14;18) translocation, leading to the fusion of the BCL2 gene with the IGH locus. Deregulated synthesis of the anti-apoptotic Bcl-2 protein leads to clonal proliferation.
Presentation: Extensive lymphadenopathy and splenomegaly. • Patients are often asymptomatic.
Histopathology Nodal architecture is supplanted by contiguous neoplastic follicles. Neoplastic follicles are devoid of mantle zones and consist of haphazardly arranged neoplastic centroblasts and centrocytes. Tangible body macrophages are typically lacking. • Neoplastic cells typically exhibit interfollicular dissemination. Bone marrow involvement is defined by paratrabecular clusters of malignant centrocytes and centroblasts. Immunophenotype PAX5, CD20, and CD79a, which are B-cell markers, exhibit positivity. Bcl-2, Bcl-6, and CD10 are furthermore positive. • CD5, CD23, and cyclin D1 are absent.
Prognosis is contingent upon the severity of the disease and the grade of the tumor. Approximately 25% progression into a high-grade lymphoma, typically diffuse large B-cell lymphoma, correlating with a swift clinical deterioration and mortality.
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Pathology - Lichen planus
Definition • An inflammatory dermatosis characterized by pruritic purple papules clinically and a lichenoid response pattern histologically.
Epidemiology • Affects around 1% of the population. • Typically occurs in middle-aged people, with a slight prevalence in females. Aetiology: Unknown. Pathogenesis • Believed to signify a delayed-type hypersensitivity response to an unidentified epidermal antigen.
Presentation • The cutaneous lesions are diminutive, flat-topped, violaceous papules that are typically associated with acute pruritus. • Delicate white lines (Wickham’s striae) typically traverse the surface. • The lesions predominantly manifest on the flexor surfaces of the wrists, the extensor surfaces of the hands, and the forearms. • Oral involvement is prevalent (see p. 86), as are genital lesionsespecially in males.
Histopathology • A substantial band-like inflammatory infiltrate comprising lymphocytes and macrophages is observed beneath the epidermis. • The basal layer of the epidermis exhibits vacuolar damage accompanied by cytoid body formation and melanin leakage. • The epidermis demonstrates irregular acanthosis, hyperkeratosis, and wedge-shaped hypergranulosis.
Prognosis: In the majority of instances, the condition resolves autonomously over a variable duration ranging from weeks to one year.
Definition • An inflammatory dermatosis characterized by pruritic purple papules clinically and a lichenoid response pattern histologically.
Epidemiology • Affects around 1% of the population. • Typically occurs in middle-aged people, with a slight prevalence in females. Aetiology: Unknown. Pathogenesis • Believed to signify a delayed-type hypersensitivity response to an unidentified epidermal antigen.
Presentation • The cutaneous lesions are diminutive, flat-topped, violaceous papules that are typically associated with acute pruritus. • Delicate white lines (Wickham’s striae) typically traverse the surface. • The lesions predominantly manifest on the flexor surfaces of the wrists, the extensor surfaces of the hands, and the forearms. • Oral involvement is prevalent (see p. 86), as are genital lesionsespecially in males.
Histopathology • A substantial band-like inflammatory infiltrate comprising lymphocytes and macrophages is observed beneath the epidermis. • The basal layer of the epidermis exhibits vacuolar damage accompanied by cytoid body formation and melanin leakage. • The epidermis demonstrates irregular acanthosis, hyperkeratosis, and wedge-shaped hypergranulosis.
Prognosis: In the majority of instances, the condition resolves autonomously over a variable duration ranging from weeks to one year.
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Pathology- Psoriasis
Definition • A chronic, recurrent dermatological condition characterized by aberrant hyperproliferation of the epidermis. Epidemiology • Prevalent, impacting approximately 2% of the population. • Average age of onset is 25 years.
Aetiology Current evidence indicates that psoriasis arises from an aberrant immune response to an environmental trigger in a genetically predisposed individual. • Factors recognized to provoke or intensify the disease encompass stress, infections, climate, alcohol consumption, smoking, and trauma. Genome-wide linkage study has identified a minimum of nine chromosomal loci linked to psoriasis, predominantly comprising genes that encode HLA proteins, cytokines, or cytokine receptors.
Pathogenesis Activated plasmacytoid dendritic cells in the skin travel to draining lymph nodes, where they facilitate the differentiation of naïve T-cells into type 1 and type 17 helper and cytotoxic T-cells. Effector T-cells migrate to the skin, where they produce cytokines such as IL-17, IL-22, IFN-γ, and TNF-α, which promote the hyperproliferation of epidermal keratinocytes. The typical presentation of psoriasis features well-defined erythematous oval plaques adorned with adhering silvery scales. The preferred locations are the elbows, knees, and scalp. Nail involvement frequently presents with pitting and onycholysis.
Guttate psoriasis is a clinical variation distinguished by tiny, erythematous papules measuring 1–5mm in size. Numerous instances are preceded by streptococcal infection. Severe psoriasis can lead to erythroderma (erythrodermic psoriasis).
Histopathology Typical lesions exhibit psoriasiform epidermal proliferation accompanied by thinning of the suprapapillary plates. Plaques of parakeratosis exhibit a reduction of the granular layer underlying the parakeratosis. Collections of neutrophils are observed in the stratum corneum (Munro microabscesses) and may also be present inside the stratum spinosum. The dermis comprises dilated capillaries and an inflammatory infiltrate. Prognosis • Typically follows a chronic trajectory. • Can substantially affect quality of life.
Definition • A chronic, recurrent dermatological condition characterized by aberrant hyperproliferation of the epidermis. Epidemiology • Prevalent, impacting approximately 2% of the population. • Average age of onset is 25 years.
Aetiology Current evidence indicates that psoriasis arises from an aberrant immune response to an environmental trigger in a genetically predisposed individual. • Factors recognized to provoke or intensify the disease encompass stress, infections, climate, alcohol consumption, smoking, and trauma. Genome-wide linkage study has identified a minimum of nine chromosomal loci linked to psoriasis, predominantly comprising genes that encode HLA proteins, cytokines, or cytokine receptors.
Pathogenesis Activated plasmacytoid dendritic cells in the skin travel to draining lymph nodes, where they facilitate the differentiation of naïve T-cells into type 1 and type 17 helper and cytotoxic T-cells. Effector T-cells migrate to the skin, where they produce cytokines such as IL-17, IL-22, IFN-γ, and TNF-α, which promote the hyperproliferation of epidermal keratinocytes. The typical presentation of psoriasis features well-defined erythematous oval plaques adorned with adhering silvery scales. The preferred locations are the elbows, knees, and scalp. Nail involvement frequently presents with pitting and onycholysis.
Guttate psoriasis is a clinical variation distinguished by tiny, erythematous papules measuring 1–5mm in size. Numerous instances are preceded by streptococcal infection. Severe psoriasis can lead to erythroderma (erythrodermic psoriasis).
Histopathology Typical lesions exhibit psoriasiform epidermal proliferation accompanied by thinning of the suprapapillary plates. Plaques of parakeratosis exhibit a reduction of the granular layer underlying the parakeratosis. Collections of neutrophils are observed in the stratum corneum (Munro microabscesses) and may also be present inside the stratum spinosum. The dermis comprises dilated capillaries and an inflammatory infiltrate. Prognosis • Typically follows a chronic trajectory. • Can substantially affect quality of life.
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Pathology – Eczemas
Definition • A collection of inflammatory dermatoses clinically identified by an erythematous papulovesicular eruption and histologically defined by intraepidermal edema (spongiosis).
Atopic dermatitis • Chronic dermatitis prevalent in individuals with atopy. • A highly prevalent condition with incidence rates reaching up to 15%. • Generally manifests in newborns and children. • Clinically induces a pruritic erythematous papulovesicular eruption affecting the facial region and the extensor surfaces of the upper and lower limbs. Biopsies from acute lesions reveal epidermal spongiosis and dermal inflammation. Biopsies from subsequent lesions demonstrate epidermal thickening and hyperkeratosis accompanied by moderate spongiosis.
Contact dermatitis due to irritants • Inflammatory dermatosis induced by the direct toxic impact of an irritant. • A prevalent etiology of occupational dermatosis. • Clinically induces erythema accompanied by vesiculation. Biopsies reveal epidermal spongiosis and dermal inflammation.
Allergic contact dermatitis • Inflammatory dermatosis resulting from a delayed-type hypersensitivity response to an allergen to which the patient has been exposed. • A prevalent occupational dermatosis, notably documented among hairdressers. • Clinically induces pruritic papules and vesicles 12–48 hours post-exposure. • Frequent offenders comprise nickel, cosmetics, and edibles. Biopsies reveal epidermal spongiosis accompanied by vesicle development and an inflammatory infiltrate, typically including eosinophils.
Nummular dermatitis • Inflammatory dermatosis of indeterminate etiology. • Clinically presents as small papules and vesicles that merge into coin-shaped plaques. • Biopsies reveal epidermal spongiosis and inflammation in first lesions. Advanced lesions exhibit epidermal hyperplasia.
Seborrheic dermatitis • Prevalent inflammatory dermatological condition impacting 1–3% of the population. Evidence indicates it may stem from an atypical immunological response to Malassezia organisms, though this remains contentious. • Clinically presents with erythematous, scaly papules and plaques, occasionally exhibiting a greasy look, located on the scalp, ears, eyebrows, and nasolabial region. Biopsies reveal varied epidermal spongiosis and hyperplasia accompanied by overlaying parakeratosis centered on hair follicles.
Definition • A collection of inflammatory dermatoses clinically identified by an erythematous papulovesicular eruption and histologically defined by intraepidermal edema (spongiosis).
Atopic dermatitis • Chronic dermatitis prevalent in individuals with atopy. • A highly prevalent condition with incidence rates reaching up to 15%. • Generally manifests in newborns and children. • Clinically induces a pruritic erythematous papulovesicular eruption affecting the facial region and the extensor surfaces of the upper and lower limbs. Biopsies from acute lesions reveal epidermal spongiosis and dermal inflammation. Biopsies from subsequent lesions demonstrate epidermal thickening and hyperkeratosis accompanied by moderate spongiosis.
Contact dermatitis due to irritants • Inflammatory dermatosis induced by the direct toxic impact of an irritant. • A prevalent etiology of occupational dermatosis. • Clinically induces erythema accompanied by vesiculation. Biopsies reveal epidermal spongiosis and dermal inflammation.
Allergic contact dermatitis • Inflammatory dermatosis resulting from a delayed-type hypersensitivity response to an allergen to which the patient has been exposed. • A prevalent occupational dermatosis, notably documented among hairdressers. • Clinically induces pruritic papules and vesicles 12–48 hours post-exposure. • Frequent offenders comprise nickel, cosmetics, and edibles. Biopsies reveal epidermal spongiosis accompanied by vesicle development and an inflammatory infiltrate, typically including eosinophils.
Nummular dermatitis • Inflammatory dermatosis of indeterminate etiology. • Clinically presents as small papules and vesicles that merge into coin-shaped plaques. • Biopsies reveal epidermal spongiosis and inflammation in first lesions. Advanced lesions exhibit epidermal hyperplasia.
Seborrheic dermatitis • Prevalent inflammatory dermatological condition impacting 1–3% of the population. Evidence indicates it may stem from an atypical immunological response to Malassezia organisms, though this remains contentious. • Clinically presents with erythematous, scaly papules and plaques, occasionally exhibiting a greasy look, located on the scalp, ears, eyebrows, and nasolabial region. Biopsies reveal varied epidermal spongiosis and hyperplasia accompanied by overlaying parakeratosis centered on hair follicles.
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Pathology - Mantle cell lymphoma
A mature B-cell neoplasm characterized by monomorphic, small to medium-sized lymphoid cells exhibiting uneven nuclear outlines and a CCND1 translocation.
Epidemiology • Constitutes 3–10% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults, with a mean age of 60 years.
Aetiology: Unknown. Genetics • Nearly all instances exhibit a t(11;14) translocation involving the CCND1 (cyclin D1) and IGH genes. Deregulated expression of cyclin D1 leads to unrestrained proliferation of lymphoid cells.
Presentation: The majority of individuals exhibit lymph node involvement. The liver, spleen, bone marrow, or peripheral blood may also be affected. Extranodal areas, especially the gastrointestinal tract, may also be involved. Histopathology • Affected tissues are substituted by sheets of monomorphic, tiny to medium-sized lymphoid cells exhibiting irregular nuclear contours. • Hyalinized small blood arteries and dispersed epithelioid histiocytes are frequently observed. Immunophenotype: B-cell markers PAX5, CD20, and CD79a exhibit positivity. • CD5 and cyclin D1 exhibit positivity. • CD23 and CD10 are often negative.
Prognosis • Despite its unremarkable look, the prognosis is typically unfavorable, with a median survival of merely 3–5 years.
A mature B-cell neoplasm characterized by monomorphic, small to medium-sized lymphoid cells exhibiting uneven nuclear outlines and a CCND1 translocation.
Epidemiology • Constitutes 3–10% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults, with a mean age of 60 years.
Aetiology: Unknown. Genetics • Nearly all instances exhibit a t(11;14) translocation involving the CCND1 (cyclin D1) and IGH genes. Deregulated expression of cyclin D1 leads to unrestrained proliferation of lymphoid cells.
Presentation: The majority of individuals exhibit lymph node involvement. The liver, spleen, bone marrow, or peripheral blood may also be affected. Extranodal areas, especially the gastrointestinal tract, may also be involved. Histopathology • Affected tissues are substituted by sheets of monomorphic, tiny to medium-sized lymphoid cells exhibiting irregular nuclear contours. • Hyalinized small blood arteries and dispersed epithelioid histiocytes are frequently observed. Immunophenotype: B-cell markers PAX5, CD20, and CD79a exhibit positivity. • CD5 and cyclin D1 exhibit positivity. • CD23 and CD10 are often negative.
Prognosis • Despite its unremarkable look, the prognosis is typically unfavorable, with a median survival of merely 3–5 years.
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Pathology - Primary amyloidosis
Definition: A plasma cell neoplasm characterized by the accumulation of AL amyloid in several tissues.
Epidemiology • Uncommon ailment. The median age at diagnosis is 65 years, with a predominance of males.
Aetiology The majority of patients possess an underlying plasma cell neoplasm yet do not fulfill the diagnostic criteria for plasma cell myeloma. Pathogenesis • AL amyloid consists of immunoglobulin light chains produced by monoclonal plasma cells, which aggregate in diverse tissues in a β-pleated sheet configuration. • The deposited amyloid comprises both intact light chains and fragments of the variable NH2-terminal region.
Presentation • Clinical manifestations associated with amyloid accumulation in several organs. Commonly affected areas encompass the skin, kidneys, heart, liver, intestines, and peripheral nerves. Characteristic manifestations include purpura, peripheral neuropathy, cardiac insufficiency, nephrotic syndrome, and malabsorption.
Histopathology • Amyloid can be identified in several tissues as a pink, amorphous material. The Congo Amyloid exhibits red staining under ordinary light microscopy and 'apple green' under polarized light. Bone marrow biopsies generally reveal a little elevation in plasma cells, which may present as either normal or abnormal. The plasma cells exhibit monotypism for either kappa or lambda light chains.
Prognosis: • Dismal prognosis with a median survival of merely 2 years from diagnosis. The predominant cause of mortality is amyloid-related heart failure.
Definition: A plasma cell neoplasm characterized by the accumulation of AL amyloid in several tissues.
Epidemiology • Uncommon ailment. The median age at diagnosis is 65 years, with a predominance of males.
Aetiology The majority of patients possess an underlying plasma cell neoplasm yet do not fulfill the diagnostic criteria for plasma cell myeloma. Pathogenesis • AL amyloid consists of immunoglobulin light chains produced by monoclonal plasma cells, which aggregate in diverse tissues in a β-pleated sheet configuration. • The deposited amyloid comprises both intact light chains and fragments of the variable NH2-terminal region.
Presentation • Clinical manifestations associated with amyloid accumulation in several organs. Commonly affected areas encompass the skin, kidneys, heart, liver, intestines, and peripheral nerves. Characteristic manifestations include purpura, peripheral neuropathy, cardiac insufficiency, nephrotic syndrome, and malabsorption.
Histopathology • Amyloid can be identified in several tissues as a pink, amorphous material. The Congo Amyloid exhibits red staining under ordinary light microscopy and 'apple green' under polarized light. Bone marrow biopsies generally reveal a little elevation in plasma cells, which may present as either normal or abnormal. The plasma cells exhibit monotypism for either kappa or lambda light chains.
Prognosis: • Dismal prognosis with a median survival of merely 2 years from diagnosis. The predominant cause of mortality is amyloid-related heart failure.
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Pathology - Plasma cell myeloma
Definition: A diffuse neoplasm of plasma cells originating from bone marrow, characterized by the presence of paraproteins in serum and/or urine.
Epidemiology • Incidence ranges from 3 to 5 per 100,000 individuals. •
Manifestations are observed in older persons, with a mean diagnostic age of 70 years. • There is a masculine predominance of 1.5:1.
Aetiology • Unidentified.
Pathogenesis The neoplastic plasma cells produce cytokines that activate osteoclasts, resulting in lytic bone lesions. Circulating paraprotein inhibits normal immunoglobulin synthesis, hence elevating the susceptibility to infections. Free light chains traversing the kidneys contribute to renal failure.
Presentation: • Ostealgia and recurring infections. • Anemia, elevated ESR, hypercalcemia, and renal dysfunction are prevalent.
Histopathology A definitive diagnosis necessitates a bone marrow biopsy. • The bone marrow exhibits an abundance of monoclonal plasma cells organized in clusters, nodules, or sheets. • Clonality can be confirmed immunohistochemically by demonstrating kappa or lambda light chain restriction.
Prognosis • Myeloma is an incurable condition. • Average survival is 3–4 years post-diagnosis.
Definition: A diffuse neoplasm of plasma cells originating from bone marrow, characterized by the presence of paraproteins in serum and/or urine.
Epidemiology • Incidence ranges from 3 to 5 per 100,000 individuals. •
Manifestations are observed in older persons, with a mean diagnostic age of 70 years. • There is a masculine predominance of 1.5:1.
Aetiology • Unidentified.
Pathogenesis The neoplastic plasma cells produce cytokines that activate osteoclasts, resulting in lytic bone lesions. Circulating paraprotein inhibits normal immunoglobulin synthesis, hence elevating the susceptibility to infections. Free light chains traversing the kidneys contribute to renal failure.
Presentation: • Ostealgia and recurring infections. • Anemia, elevated ESR, hypercalcemia, and renal dysfunction are prevalent.
Histopathology A definitive diagnosis necessitates a bone marrow biopsy. • The bone marrow exhibits an abundance of monoclonal plasma cells organized in clusters, nodules, or sheets. • Clonality can be confirmed immunohistochemically by demonstrating kappa or lambda light chain restriction.
Prognosis • Myeloma is an incurable condition. • Average survival is 3–4 years post-diagnosis.
