Pathologie (Fach) / Hämolymphopoetisches System (Lektion)

In dieser Lektion befinden sich 25 Karteikarten

6. SJ.

Diese Lektion wurde von estoffel erstellt.

Lektion lernen

zurück  |  weiter 1 / 1

  • Retikulozytär-abszedierende Lymphadenitis 1. Basophile Nekrosezonen durchsetzt von neutrophilen Granulozyten (abszedierende Entzündung) 2. Demarkierung der Abszesse durch palisadenförmig angeordnete epitheloide Histiozyten (heller Randwall um die Abszesse). 3. Vereinzelt mehrkernige Riesenzellen im histiozytären Randwall. 4. Vernarbte verdickte Lymphknotenkapsel. Differentialdiagnose:- Katzenkrankheit- Lymphogranuloma venereum- Eitrige Lymphadenitis- Tuberkulose- Sarkoidose - Brucellose- Tularemia
  • Sarcoidosis Multisystem disorder characterized by non-caseating granulomatous inflammation. - Most common among African-American females T cells and macrophages get attracted to a specific spot (most likely hilar lymph nodes) and form granulomas. T-cells at the periphery and macrophages in the center. Noncaseating → no necrosis at the center.Macrophages fuse together to form epithelioid cells and Langhans giant cells. Within the Langhans cell are Schaumann bodies (calcium & protein deposits) and asteroid bodies (look like tiny stars). - Generalized symptoms: Fever, weight loss, fatigue- Pulmonary: dyspnea, cough, chest pain- Tender skin nodules: Erythema nodosum, typically on lower leg (inflammation of fat in the skin)- Lupus pernio: purple skin lesions on the nose, cheeks, chin and/or ears- Uveitis- Heart: Restrictive cardiomyopathy, pericardial effusion, rrhythmias Diagnosis:- CT-Scan: Bilateral hilar lymphadenopathy- ↑ Calcium (excess D from macrophages), ↑ ACE (from T-cells)- Bronchoalveolar lavage: increased CD4+/CD8+ ratio- Biopsy Treatment:- Mild → none, resolves spontaneously within weeks- Severe → steroids
  • Langhans giant cell - Differential The multi nucleated giant cells (Langhans type) originate from fused epithelioid cells (macrophages). They are part of epithelioid cell granulomas. These are found for example in infectious diseases such as:- Tuberculosis (caseating)- Leprosy- Sarcoidosis- Crohn's disease- Temporal arteritis- Rheumatoid arthritis Characteristic is the horseshoe shaped arrangement of the numerous nuclei. The Langhans Giant Cell is named after the Director of the Institute of Pathology at the University of Bern, Theodor Langhans (1839-1915), who in 1868 coined the term 'Giant Cell'.
  • Differential diagnosis of granulomatous disease Sarcoidosis- Non-caseating granuloma- Giant cells Tuberculosis (TB)- Caseating granulomas- Polynuclear Langhans giant cells, epithelioid macrophages, and lymphocytes- Acid-fast M. tuberculosis Hodgkin's lymphoma- Non-caseating granuloma- Reed-Sternberg cells- Inflammatory cell infiltrate (e.g., Eosinophils, fibroblasts, plasma cells) Non-Hodgkin's lymphoma Non-caseating granuloma without Reed-Sternberg cells- B-lymphocytes or T-lymphocytes- Inflammatory cell infiltrate Pneumoconiosis- Non-caseating granuloma- Silica/asbestos bodies Granulomatosis with polyangiitis- Non-caseating granuloma Histoplasmosis- Non-caseating granuloma- Identification of H. capsulatum yeast with silver stain
  • Sarkoidose 1. Wenig erhaltenes Lymphknotengewebe 2. Granulome aus Epitheloidzellen (längsovale Kerne). 3. Riesenzellen.- Gelegentlich Schaumann-Körper (Kalkkörper)- Gelegentlich Asteroid-Körper (Sterne) 4. Perigranulomatöse Fibrose.
  • Miliartuberkulose 1. Milzstruktur erkennbar an periarteriolären Lymphscheiden 2. Hyperämie der roten Pulpa 3. Zahlreiche, teilweise zentral verkäsende epitheloid-riesenzellige Granulome 4. Intrazelluläre Vermehrung von säurefesten Stäbchen in Histiozyten
  • Tuberculosis Common infectious disease (2 billion people infected worldwide) that is caused by Mycobacterium tuberculosis and typically affects the lungs.- Countries with the highest incidence: India, Indonesia, China, Nigeria, Pakistan, and South Africa Mycobacteria: Nonmotile, aerobic, gram-positive, acid-fast bacilli with a rich lipid cell wall.- Mycobacterium tuberculosis: 95% of cases- Mycobacterium bovis: Common pathogen causing gastrointestinal tuberculosis Primary tuberculosis (primary infection)- Latent tuberculosis infection (LTBI): → Primary infection without any pathological findings on radiological imaging; however, screening tests indicating previous infection with M. tuberculosis are positive. → The lifetime risk of reactivation TB for a person with LTBI is about 5–10%.- Active primary tuberculosis (1–5% of cases): → Primary infection with radiological-pathological findings of tuberculosis (eg, Ghon complex) M. tuberculosis remains dormant within the host and may be reactivated once the immune system becomes compromised (e.g., by high doses of glucocorticoids or chemotherapeutic agents, HIV infection)! Reactivation tuberculosis (secondary infection)- Following a latent primary TB infection- 80% of secondary infections begin in the lungs  Treatment:Active disease:- Initiation phase: 2 months of isoniazid + rifampin + pyrazinamide + ethambutol- Continuation phase: 4 months of isoniazid + rifampinLatent infection:- Isoniazid monotherapy for 9 month
  • Hodgkin lymphoma Malignant lymphoma that is typically of B-cell origin. - Bimodal distribution (1st peak: 25-30 years, 2nd peak: 50-70 years)- Male predominance, except in nodular sclerosing type Etiology:- Association with EBV, immunodeficiency (eg HIV), autoimmune diseases (eg, rheumatoid arthritis, sarcoidosis) Symptoms:- Painless lymphadenopathy (Cervical > axillary > inguinal), mediastinal mass, splenomegaly- B symptoms: Night sweats, weight loss > 10% in the past 6 months, fever > 38°C (100.4°F)- Pel-Ebstein fever (Intermittent fevers of 1-2 weeks)- Alcohol-induced pain (Pain in involved lymph nodes after ingestion of alcohol)- Pruritus Stages: Based on Ann-Arbor system Diagnostics:- CBC: Anemia, ↑/↓ WBC, eosinophilia- Lymph node excision:→ Reed-Sternberg cells: polynuclear giant cells originating from B cells, CD15/CD30-positive→ Hodgkin cells: mononuclear→ Inflammatory background (T cells, fibroblasts, eosinophils)→ Granuloma formation- Chest x-ray, CT, PET-CT Treatment:- Stage I, II: ABVD (adriamycin aka doxorubicin, bleomycin, vinblastine, dacarbazine) + radiation- Stage III, IV: ABVD, Stanford V, BEACOPP + radiation
  • Hodgkin lymphoma - Subgroups Differentiates between classical (95%) and lymphocyte predominant Hodgkin lymphoma (5%). Classical Hodgkin lymphoma: Do not express CD45 or CD20, express CD15 and CD301. Nodular sclerosing- Most common subtype (> 60%) with good prognosis- Localization: mostly mediastinal and cervical- Lacunar cells2. Mixed-cellularity - Up to 30% of cases; prognosis slightly worse than with nodular sclerosing subtype- Localization: mostly abdominal and splenic3. Lymphocyte-rich - Rare; best prognosis- Localization: mostly cervical and axillary4. Lymphocyte-depleted - Very rare (< 1%); bad prognosis- Localization: mostly below the diaphragm Nodular lymphocyte predominant HL (NLPHL): Express CD45 and CD20, not CD15 or CD30- Rare (5%); very good prognosis- Popcorn cells
  • Hodgkin-Lymphom, nodulär-sklerosierend 1. Lymphknoten durchzogen von aus der Kapsel einstrahlenden Kollagenfaserbünden 2. Lacunarzellen (CD15, CD30 positiv) 3. Reed-Sternbergzelle mit spiegelbildlich angeordneten Kernen. 3. Mischzellinfiltrat reaktiver Zellen: Lymphozyten, Eosinophile, Histiozyten, Plasmazellen
  • Hodgkin Lymphom, Mischzelltyp 1. Zerstörte Lymphknotenarchitektur. 2. Zahlreiche einkernige Hodgkinzellen und mehrkernige Reed-Sternbergzellen mit vergrösserten eosinophilen Nukleolen. 3. Gemischtes reaktives Infiltrat aus nicht neoplastischen Lymphozyten, Plasmazellen, epitheloiden Histiozyten und eosinophilen Granulozyten. 4. Keine Lakunarzellen, keine Fibrose.
  • Non-Hodgkin lymphoma Non-Hodgkin lymphoma is the most common hematopoietic neoplasm and make up approx. 85% of lymphomas. Etiology: - Chromosomal translocations; most commonly t(14;18)- Infections: EBV, HIV, HTLV-1, HCV, Helicobacter pylori → MALT gastric lymphoma- Autoimmune diseases: Hashimoto thyroiditis, rheumatic disease- Immunodeficiency: congenital immunodeficiencies, AIDS, history of chemotherapy and/or immunosuppressive therapy B-cell lymphomas (85%)Low-grade:- Follicular lymphoma- Small lymphocytic lymphoma- Hairy cell leukemia- MALT lymphoma- Lymphoplasmocytic lymphoma (Waldenström macroglobulinemia)High-grade:- Burkitt lymphoma- Diffuse large B-cell lymphoma- Mantel cell lymphoma T-cell lymphoma:- Mycosis fungoides → Sézary syndrome- Adult T-cell lymphoma Treatment: - Most NHL: CHOP regimen (Cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine, prednisolone)- B-cell NHL: R-CHOP (combination of CHOP and CD20 antibody rituximab)- Hairy cell leukemia: cladribine or pentostatin
  • Mediastinal mass 1. Thymoma 2. Teratoma (and other germ cell tumors) 3. Thyroid neoplasm 4. Terrible lymphoma
  • Follicular lymphoma - Most common low-grade lymphoma in adults - Slowly progressive, painless lymphadenopathy of alternating size (“waxing and waning”) and splenomegaly - Translocation t(14,18): heavy-chain Ig (14) and Bcl-2 (18) are translocated, resulting in an overexpression of Bcl-2, which inhibits apoptosis Histology: Nodular, small cells with cleaved nuclei
  • Follikuläres Non-Hodgkin Lymphom (zentroblastisch-zentrozytisch) 1. Zerstörung der Lymphknotenarchitektur. 2. Dichtstehende monotone neoplastische Follikel in Rinde und Mark (follikuläres Wachstumsmuster). 3. Monotone Follikelzentren bestehend aus kleinen Zentrozyten, follikulären dendritischen Zellen und Zentroblasten mit mehreren randständigen Nukleolen.
  • Diffus grosszelliges Non-Hodgkin Lymphom 1. Zerstörung der Lymphknotenarchitektur durch diffuses Lymphominfiltrat 2. Einzelzelle mit basophilem Zytoplasma und grossem, zentralem Nucleolus (Immunoblasten) bzw. doppeltem, randständigem Nucleolus (Zentroblasten) 3. Reichlich Mitosen und Apoptosen
  • Hairy cell leukemia - More common in middle-aged men - Similar in appearance to CLL (associated with a better prognosis) Symptoms:- Symptomatic cytopenia - Massive splenomegaly dominates- No lymphadenopathy- Rarely B symptoms- Up to 20% have leukocytosis Histology:- Hairy cells- Usually positive tartrate-resistant acid phosphatase (TRAP) stain and CD11c marker Diagnosis:- Bone marrow aspiration: often a dry tap
  • Multiple myeloma Malignant plasma cell dyscrasia characterized by uncontrolled proliferation and the diffuse infiltration of monoclonal plasma cells in the bone marrow.- Multiple myeloma: diffuse infiltration of the bone marrow- Plasmacytoma: extramedullary solitary mass that may affect bones or soft tissueMGUS usually precedes multiple myeloma. - Males > Females; peak incidence 50-70 years Classification:- IgG: 50% of multiple myelomas- IgA: 25% of multiple myelomas- Bence Jones myeloma (free light chains excreted in urine): 20% of multiple myelomas Symptoms:- Often asymptomatic- Bone pain - especially back pain (most common symptom), spontaneous fractures- Symptoms of hypercalcemia- Mild fever, night sweats, weight loss- Weakness and anemia, increased risk of infection, increased risk of petechial bleeding- Foamy urine, caused by Bence Jones proteinuria Diagnosis:- Serum protein electrophoresis (best initial test): monoclonal gammopathy with M protein- Urine protein electrophoresis: Bence Jones proteins- Bone marrow biopsy (confirmatory test)- X-ray skeletal survey: Osteolysis, multiple lytic lesions Therapy:- Asymptomatic patients: watch and wait- Hematopoetic stem cell transplantation (HCT) eligible: induction therapy followed by autologous HCT- HCT ineligible: chemotherapy alone (eg, dexamethasone and lenalidomide)
  • Plasmazellmyelom 1. Knochenmarkstanze mit nodulärer Hyperzellularität des blutbildenden Marks. 2. In den hyperzellulären Arealen dichte Rasen neoplastischer atypischer Plasmazellen.- Die Plasmazellen sind polymorph, vereinzelt mehrkernig. - Exzentrisch im Zytoplasma lokalisierte vergrösserte Radspeichenkerne.- Zellkerne teils mit vergrössertem Nukleolus. 3. Ausserhalb der Plasmazellrasen findet sich spärlich residuelles blutbildendes Knochenmark.
  • Acute myeloid leukemia (AML) Malignant neoplastic disease that arises from or myeloid cell lines. - Peak incidence: 65 years- 80% of acute leukemias during adulthood are myelogenous Etiology:- Environmental: Benzene, ionizing radiation, tobacco, chemotherapy drugs- Genetic: Down syndrome, Philadelphia translocation, t(15;17) translocation (particularly APL), Fanconi anemia- Myeloproliferative and myelodysplastic disorders: Osteomyelofibrosis, CML Classification: French-American-British (FAB): 8 subtypes Symptoms:- Fatigue, pallor, and weakness - Bleeding (petechiae, epistaxis, or hematoma)- Hepatomegaly and/or splenomegaly- Headache, visual field changes, or other CNS symptoms - Fever  Diagnostic work-up: CBC, peripheral blood smear, and a bone marrow aspirate or biopsy.- Myeloperoxidase (MPO) ⊕ Therapy: Induction therapy, consolidation therapy, maintenance therapy- Induction therapy: cytarabine and anthracyclines (eg, daunorubicin)
  • Acute lymphoid leukemia (ALL) Malignant neoplastic diseases that arise from lymphoid cell lines. - Peak incidence: 2–5 years- Most common malignant disease in children- M > F Etiology:- Adult T-cell leukemia/lymphoma is linked to infection with HTL viruses.- Genetic factors: Down syndrome, neurofibromatosis type 1, ataxia telangiectasia Classification:- B-cell ALL (80-85%): Early pre-B ALL, common ALL, pre-B ALL, mature B-ALL- T-cell ALL (15–20%): Pre-T ALL, intermediate-T ALL, mature T-cell ALL Symptoms:- Painless lymphadenopathy- Fever, night sweats, unexplained weight loss- Bone pain- Testicular enlargement (rare finding)- Airway obstruction (stridor, difficulty breathing) caused by mediastinal infiltration- Meningeal leukemia (or leukemic meningitis) → headache, neck stiffness Diagnosis: CBC, peripheral blood smear, and a bone marrow aspirate or biopsy.- TdT ⊕ Therapy: Induction therapy, consolidation therapy, maintenance therapy- Induction therapy: vincristine, glucocorticoids, asparaginase- In the case of Philadelphia chromosome mutation: BCR-ABL tyrosine kinase inhibitor (eg, imatinib)
  • Akute myeloische Leukämie (AML) 1. Ausgeprägte Hyperzellularität (>20% Blasten) mit kompletter Verdrängung des blutbildenden Marks und des Fettmarks (packed marrow). 2. Mittelgrosse Myeloblasten (12-20 μm) mit grossen rundlichen Kernen und unterschiedlich prominenten Nukleolen. 3. Schmaler agranulärer basophiler Zytoplasmasaum. 4. Zahlreiche Apoptosen. - Auer rods (Kristallizationen von Myeloperoxidase) v.a. beim AML M3 (acute promyelocytic leukemia).
  • Chronic myeloid leukemia (CML) Part of the myeloproliferative disorders.Malignancy of the hematopoietic stem cells with excessive proliferation of the myeloid lineage (especially granulocytes). Peak incidence: 50–60 years Reciprocal translocation between chromosome 9 and chromosome 22 → Philadelphia chromosome → formation of the BCR-ABL gene → BCR-ABL non-receptor tyrosine kinase with increased enzyme activity SymptomsChronic phase:- Can persist for up to 10 years and is often clinically unremarkable- Weight loss, fever, night sweats, fatigue- Splenomegaly: abdominal discomfort in the left upper quadrant- Swollen lymph nodes are not typical in CML.Accelerated phase:- Anemia, petechial bleeding, infection and fever- Extreme pleocytosis (Infarctions: splenic and myocardial infarctions, retinal vessel occlusion, leukemic priapism)- Extreme splenomegalyBlast crisis: Symptoms of AML Treatment: Tyrosine kinase inhibitors (imatinib)
  • Lymphadenopathy - Differential - HIV- Mycobacterial infection (e.g., tuberculosis)- Infectious mononucleosis- Systemic lupus erythematodes Signs of malignancy: - Rapid growth- Painlessness- Hardness/coarseness- Fixed to underlying or surrounding tissue
  • Chronic lymphocytic leukemia (CLL) Low-grade B-cell lymphoma with lymphocytic leukocytosis.- Median age at diagnosis: 70-72 years - Most common type of leukemia in adults Classification: Rai staging system- Stage 0: Isolated lymphocytosis- Stage I: + Lymphadenopathy- Stage II: + Hepatomegaly and/or splenomegaly- Stage III: + Anemia (Hb < 11 g/dL)- Stage IV: + Thrombocytopenia (< 100,000/μL) Clinical features:- Weight loss, fever, night sweats, fatigue- Painless lymphadenopathy- Hepatomegaly and/or splenomegaly may occur- Repeated infections: Severe bacterial infections (e.g., necrotic erysipelas), mycosis (candidiasis), viral infections (herpes zoster)- Symptoms of anemia and thrombocytopenia- Dermatologic symptoms: Leukemia cutis, chronic pruritus, chronic urticaria Diagnostics:- Persisting lymphocytosis with a high percentage of small mature lymphocytes- Blood smear: smudge cells (Gumprecht shadows) - Flow cytometry: detection of B-CLL immunophenotype (CD19, CD20, CD23), light chain restriction (kappa or lambda)- Bone marrow aspiration (not necessary to confirm the diagnosis):→ High percentage (> 30%) of small, mature lymphocytes→ Decreased number of myeloid progenitor cells Treatment:- Asymptomatic CLL (Rai stage 0, slow disease progression): observe and monitor disease progression- Symptomatic CLL or advanced stage (Rai stage > 0, accelerated disease progression): Chemotherapy→ If CD 20 positive: rituximab→ Targeted therapy with ibrutinib- Refractory CLL or early recurrence in fit, young patients: allogeneic stem cell transplantation Complications:- Richter's transformation: transformation into a high-grade NHL in ∼5% of cases

zurück  |  weiter 1 / 1