Section 1
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Diffuse large B-cell lymphoma
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Last updated
6 years ago
Date created
Mar 1, 2020
Cards (85)
Section 1
(50 cards)
Diffuse large B-cell lymphoma
Most common type of non-Hodgkin lymphoma in adults Alterations in Bcl-2, Bcl-6
Hemophilia C
Deficiency of factor 11/ increased PTT/ AR
Multiple myeloma: general characteristics
Monoclonal plasma cell cancer "Fried egg appearance" Cancer arises in BM and produces large amounts of IgG (55%) and IgA (25%) BM >10% monoclonal plasma cells MC primary tumor arising in ppl >40-50 years old
CD14 marker is present on what cells?
Monocyte-macrophage cell lineage cell surface marker
Burkitt lymphoma
Adolescents or young adults t(8;14)- translocation of oncogene c-myc (8) and heavy chain Ig (14) Ass. w/ EBV Endemic form: jaw lesion in African Sporadic form: pelvis or abdomen
Bite cells and Heinz bodies
G6PD deficiency
Von Willebrand Disease: Labs and Tx
Increased BT Normal platelet count Increased PTTa Tx: Desmopressin
Reed-Sternberg cells: description and markers
Giant cells, binucleate, with 2 halves as mirror images CD15 and CD30 + B-cell origin
Hodgkin lymphoma
Localized, single group of nodes Contiguous spread Many pts have relatively good prognosis Characterized by Reed- Sternberg cells Bimodal distribution (young adulthood and >55) More common in men (except for modular sclerosis type) Ass. w/ EBV
Disseminated Intravascular Coagulation (DIC): labs
Low platelets Increased bleeding time Increased PT and PTT Schistocytes Increased fibrin degradation products (D-dimers) Decreased fibrinogen
Burkitt lymphoma: histology
"Starry sky" appearance Sheets of lymphocytes with interspersed "tingle body" macrophages
Similar clinical consequence as G6PD deficiency due to inability to utilize NADPH
Glutathione reductase deficiency
Glanzmann thrombasthenia
Defect in GpIIb/IIIa/ defect in platelet aggregation
Types of Non-Hodgkin lymphoma
Neoplasms of mature B cells: -Burkitt lymphoma -Diffuse large B-cell lymphoma -Follicular lymphoma -Mantle cell lymphoma -Marginal zone lymphoma -Primary CNS lymphoma Neoplasms of mature T cells -Adult T-cell lymphoma -Mycosis fungoides/ Sezary syndrome
Factor V Leiden
Production of mutant factor 5 that is resistant to degradation by Protein C act. DNA point mutation (G to A) MCC of inherited hypercoagulability in Caucasians Complications: DVT, cerebral vein thrombosis, recurrent pregnancy loss
Hemolytic Uremic Syndrome (HUS): classic triad
Thrombocytopenia, microangiopathic hemolytic anemia, acute renal failure
Hemophilia B
Deficiency of Factor 9/ increased PTT/ X-linked recessive
Wiskott-Aldrich syndrome
Triad: eczema, recurrent infections (B and T-lymphocyte deficiency) and thrombocytopenia X-linked (more common in males) Onset is early in life (thrombocytopenia and eczema present at birth)
Multiple myeloma: px and associations
⬆️ susceptibility to infxn Primary amyloidosis Punched-out lytic bone lesions on X-ray M spike on serum protein electrophoresis Bence Jones protein (Ig light chains in urine) Rouleaux formation of RBCs on blood smear Numerous plasma cells with "clock-face" chromatin and intracytoplasmatic inclusions containing Ig think CRAB
Thrombotic Thrombocytopenic Purpura (TTP): Labs
Decreased platelet count Prolonged bleeding time Normal PT and PTT Peripheral blood smear: schistocytes Increased LDH
Follicular lymphoma
Indolent course t(14;18)- translocation of heavy chain Ig and Bcl-2 Bcl-2 inhibits apoptosis (increase in bcl-2 activity) Px w/ "waxing and waning" lymphadenopathy
Primary CNS lymphoma
Adults MC ass. w/ HIV/AIDS Considered and AIDS-defining illness Variable px: confusion, memory loss, seizures Mass lesion(s) on MRI (differential dx w/ toxoplasmosis via CSF or other lab tests)
Cells seen in Hodgkin lymphoma
Reed-Sternberg cells
Disseminated Intravascular Coagulation (DIC): causes
Sepsis (gram -) Trauma Obstetric complications Acute pancreatitis Malignancy Nephrotic syndrome Transfusion
Acute intermittent porphyria: affected enzyme?
Defective/ deficient porphobilinogen deaminase
Hemophilia A
Deficiency of factor 8/ increased PTT/ X-linked recessive
Prothrombin gene mutation
Mutation in 3' untranslated region/ increased production of prothrombin/ increased plasma levels and venous clots
Thrombotic Thrombocytopenic Purpura (TTP): symptoms
Pentad: fever, neurological symptoms (stroke), renal symptoms (renal failure), thrombocytopenia, & microangiopathic hemolytic anemia
Mycosis fungoides/ Sezary syndrome
Adults Px w/ skin patches/ plaques (cutaneous T-cell lymphoma) Characterized by atypical CD4+ cells w/ "cerebriform" nuclei May progress to Sezary syndrome (T-cell leukemia)
Immune Thrombocytopenic Purpura (ITP)
Anti-GpIIb/IIIa antibodies (IgG) against platelets Splenic macrophages destroy platelet-Ab complex No splenomegaly
Antithrombin deficiency
Inherited deficiency of antithrombin No direct effect on PT, PTT or thrombin time Diminishes the increase in PTT following heparin administration Can be acquired by RF/ nephrotic syndrome
Acute intermittent porphyria
AD inheritance Port-wine colored urine, polyneuropathy, painful abdomen, psychological disturbances, precipitated by drugs (CYP450 inducers), PBG and ALA in urine Cause: porphobilinogen (PBG) deaminase deficiency Tx: glucose and heme (inhibit ALA synthase)
Bernard-Soulier syndrome
Defect in GP1b receptor/ defect in platelet adhesion to vWF/ large platelets
t(9;22) (Philadelphia chromosome), BCR-ABL and decreased leukocyte alkaline phosphatase
Chronic myelogenous leukemia
Chronic Myelogenous Leukemia (CML)
Myeloid stem cell proliferation Dysregulated production of mature and maturing granulocytes (neutrophils, metamyelocytes, myelocytes, basophils) Low LAP Tx: Imatinib (bcr-abl TK -)
Disseminated Intravascular Coagulation (DIC)
Widespread activation of clotting➡️consumption and deficiency in clotting factors and platelets ➡️hemorrhage always secondary to another disorder
Hemolytic uremic syndrome: classic presentation and etiology
Seen in children Accompanied by bloody diarrhea Caused by shiga toxin-producing E. coli (STEC) ex. O157:H7 (Child goes to bbq and eats uncooked hamburger)
Non-Hodgkin lymphoma
Multiple lymph nodes involved, extranodal involvement common, noncontiguous spread Majority involve B cells; few are of T-cell lineage Can occur in children and adults May be ass. w/ HIV and AI diseases
Thrombotic Thrombocytopenic Purpura (TTP)
-/deficiency of ADAMTS13 (vWF metalloprotease) Decreased degradation of vWF multimers Increased large vWF multimers Increased platelet adhesion, increased platelet aggregation and thrombosis
Hemolytic anemia and jaundice secondary to increased oxidative stress due to lack of NADPH
Glucose 6-phosphate dehydrogenase deficiency (G6PD deficiency)
Immune Thrombocytopenic Purpura (ITP): 2 types
Acute ITP- seen in children ass. w/ viral infxn and is self-limited Chronic- ITP- usually seen in women in childbearing years/ may be 1st manifestation of SLE
Protein C or S deficiency
Decreased ability to inactivate factor 5a and 8a Increased risk of thrombotic skin necrosis with hemorrhage after admin of warfarin
Subtypes of Hodgkin lymphoma
Nodular sclerosis (MC) Lymphocyte rich (best prognosis) Mixed cellularity (eosinophilia, seen in immunocompromised pts) Lymphocyte depleted (seen in immunocompromised pts)
Mantle cell lymphoma
Adult males t(11;14)- translocation of heavy chain IG and cyclin D1 Very agressive, pts typically px with late-stage disease
What are the constitutional "B" sign/symptoms? Where are they seen?
Low-grade fever, night sweats, weight loss May be seen in Hodgkin and Non-Hodgkin lymphoma
Adult T-cell lymphoma
Adults Caused by HTLV infection (ass. w/ IV drug abuse) Adults px w/ cutaneous lesions Esp. affects populations in Japan, W. Africa and Caribbean Lytic bone lesions, hypercalcemia
Porphyria cutanea tarda: affected enzyme?
uroporphyrinogen decarboxylase
Von Willebrand Disease
Deficient or qualitative defect of vWF Mild Factor 8 def (intrinsic pathway defect) Autosomal dominant Abnormal ristocetin test (no platelet aggregation)
Immune Thrombocytopenic Purpura (ITP): Tx
Corticosteroids, IV IG, splenectomy (for refractory ITP)
Porphyria cutanea tarda
Blistering cutaneous photosensitivity
Section 2
(35 cards)