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Path 1 Block 3 Pt 1

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ptheodore's version from 2016-07-17 20:37

Immune Hemolytic Anemia

Question Answer
Autoimmune disorders (SLE), Lymphoid neoplasms (Chronic Lymphocytic Leukemia), Drugs (Penicillin, α-methyl dopa)Warm Antibody Type (IgG Ab active at 37°C)
Mycoplasmal infection, Inf monoCold agglutinin type (IgM Ab active below 37°C)
Extravascular hemolysis (spleen)Warm Antibody Type (IgG Ab active at 37°C)
Similar to Hereditary SpherocytosisWarm Antibody Type (IgG Ab active at 37°C)
Intravascular; schistocytes (Fragmented RBC’s)Microangiopathic hemolytic anemia
Platelet thrombi; hemolytic uremic syndrome (HUS)Microangiopathic hemolytic anemia
Platelet thrombi; thrombotic thrombocytopenic purpura (TTP)Microangiopathic hemolytic anemia
Fibrin thrombi; disseminated intravascular coagulation (DIC)Microangiopathic hemolytic anemia
Fibrin thrombi; HELLP synd. (Hemolytic, Elevated transaminases, Low platelets, associated with preeclampsia)Microangiopathic hemolytic anemia
Aortic StenosisMicroangiopathic hemolytic anemia
SchistocytesMicroangiopathic hemolytic anemia
Hemolytic anemia, unconjugated hyperbilirubinemia, kernicterusRh- incompatibility
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Lymph Node Drainage & Metastases

Question Answer
Head, neck tumors, Dental, tonsilsCervical Ln
Scalp infection Occipital Ln
Conjunctival infections, cat-scratch diseasePre-auricular Ln
ToxoplasmosisPosterior cervical Ln
STD’s & micro organisms from people who don't wear shoes.Inguinal Ln
Metastases from squamous cell ca. floor of the mouthSubmental Ln
Stomach, pancreas cancers, prostate & testicularVirchow’s node (left supraclavicular)
Breast cancersAxillary Ln
Lung cancersHilar Ln
Lung cancers, Hodgkin lymphoma (nodular sclerosing), T- cell ALLMediastinal Ln
Testicular, Burkitt lymphomaPara-aortic Ln
Vulva, penis cancersInguinal Ln
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WBC 1

Question Answer
Severe life threatening infections: lungs, kidney deep fungal infections: Candida and AspergillusNeutropenia & Agranulocytosis
Basophilia & cancerChronic Myelogenous Leukemia
TB elicits what type of cell reactionsMonocytois & Lymphocytosis
Bordetella Pertussis & TyphoidLymphocytosis
↑ Lap Score, TWBC> 50K, Shift to the left (more than 10% bands in peripheral blood smear)Leukemoid Reaction
Nucleated red cell, bone marrow infiltration (amyloidosis, Gaucher’s, leukemias, metastatic cancers)Leukoerythroblastic
Found in infections, Seen along with toxic granulationsDohle Body (light blue smudge within mature neutrophils)
Fever, pharyngitis and lymphadenopathy, ‘glandular feverInfectious mononucleosis (EBV)
Infects B-lymphocytes expressing CD21. Can activate NF-kBInfectious mononucleosis (EBV)
Positive antiviral capsid antigen test Infectious mononucleosis (EBV)
Risk of splenic ruptureInfectious mononucleosis (EBV)
Tingible body ‘macrophages’Follicular hyperplasia
Acute mesenteric lymphadenitisMimic appendicitis
Associated with; rheumatoid arthritis, toxoplasmosis, early stages of HIVFollicular hyperplasia
Tingible body macrophages (numerous macrophages containing numerous phagocytosed apoptotic bodies)Follicular hyperplasia
characterized by distension and prominence of lymphoid sinusoidsSinus histiocytosis
These changes are seen associated with lymph nodes draining cancersSinus histiocytosis
Widening of lymphatic sinusoidsSinus histiocytosis
Associated with; immunologic reactions induced by drugs (Dilantin), acute viral infections (infectious mononucleosis), vaccination against certain virusesParacortical lymphoid hyperplasia
Expansion of the paracortical/interfollicular regionsParacortical lymphoid hyperplasia
Associated with chronic inflammatory states such as Hashimoto thyroiditis, Sjogren syndrome, and H pylori gastritis.Marginal zone Lymphomas (NHL)
CD20, CD23 +, and CD5 +,tdT(-)Diffuse Large B-cell Lymphoma
CD20, CD5+, tdT+Acute Lymphocytic Leukemia
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Translocations

Question Answer
CCND1 (cyclin D1) mutations causest(11;14) involving IgH, Mantle cell lymphoma
t(11;14)Mantle cell lymphoma ( IgH & Cyclin D1)
t(9;22) (Philadelphia chromosome)Chronic Myeloid Leukemia & B-Cell Acute Lymphoblastic Leukemia
t(14;18)Follicular B-Cell Lymphoma (NHL)
t(8;􀀀 14)􀀀Burkitt Lymphoma
t(12;21)Acute􀀁 Lymphoblastic􀀁 Leukemia
t(15;17)Acute proMyelocytic Leukemia (M3)-AML
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WBC 2

Question Answer
What does Acute Lymphoblastic Leukemia (ALL), Acute Myelogenous Leukemia (AML) & Myelodysplastic Syndrome have in commonDown Syndrome
Helicobacter pyloriGastric B- cell lymphoma (MALToma)
The presence of nucleoli, and the fact that “blasts” are much larger (compare wit the red cells).Acute Lymphoblastic Leukemia
Most common childhood malignancy. Group of neoplasms composed of immature precursor B (pre B) or T (pre T) lymphocytesAcute Lymphoblastic Leukemia
NOTCH1T-cell type Acute Lymphoblastic Leukemia
PAX5 mutation, t(12;21)Acute Lymphoblastic Leukemia (B-Type)
t(9; 22) is associated withCML & B-Cell Acute Lymphoblastic Leukemia
Myeloperoxidase & Sudan Black PositiveAcute Myelogenous Leukemia
PAS +, kids 2 and 5 yearsAcute Lymphoblastic Leukemia
TdT +, CD10 (CALLA), CD19, PAX5Acute Lymphoblastic Leukemia (Pre-B Cell)
TdT+, CD2, CD3, CD4, CD7 Acute Lymphoblastic Leukemia (Pre-T Cell)
serum lactate dehydrogenase (LDH): ↑ & serum uric acid: ↑Acute Lymphoblastic Leukemia
CNS & testes are regarded as ‘sanctuary’ sitesAcute Lymphoblastic Leukemia
Anemia, neutropenia and thrombocytopenia. Typical features include, abrupt stormy onset, bone marrow depression, bone pain and tenderness, generalized lymphadenopathy, CNS manifestationsAcute Lymphoblastic Leukemia
Anterior mediastinal lymphadenopathyAcute Lymphoblastic Leukemia (T-Cell)
Tumor of hematopoietic progenitors (to accumulation of immature myeloid blasts in bone marrow)Acute Myelogenous Leukemia
What can you get secondary to Myelodysplastic syndrome (MDS)Acute Myelogenous Leukemia
Radiation exposure, chronic benzene exposure, chemotherapeutic drugs, smoking, Down synd, Bloom synd, Fanconi anemiaAcute Myelogenous Leukemia
t(15;17)Acute proMyelocytic Leukemia (M3)-AML
Responds to all-trans retinoic acid (ATRA)Acute proMyelocytic Leukemia (M3)-AML
Auer rods & Disseminated intravascular coagulation (DIC)Acute proMyelocytic Leukemia (M3)-AML
Cutaneous and gingival infiltration, positive for non-specific esterase (NSE)Acute monocytic leukemia (M5)
Acute Megakaryocytic leukemia (M7)Platelet Specific antibodies & Down Syndrome
Vitamin A derivative is used to treatAcute proMyelocytic Leukemia (M3)-AML
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WBC 3

Question Answer
Lymphocytosis, ‘smudge cells’, older than 60. Clonal B cells arrested in the B-cell differentiation pathway, intermediate between pre-B cells and mature B cellsChronic Lymphocytic Leukemia
CD19, CD20, CD5, and CD23 are positiveChronic Lymphocytic Leukemia
Hypogammaglobulinemia, autoimmune hemolytic anemia (AIHA) or thrombocytopeniaChronic Lymphocytic Leukemia
Richter syndrome; rapidly enlarging mass within lymph node/spleen, which is a “diffuse large B cell lymphoma”Chronic Lymphocytic Leukemia
Transformation of Chronic Lymphocytic Leukemia into aggressive lymphomaRichter syndrome
Activating point mutation in BRAF & overexpression of cyclin D1 (cell cycle regulator)Hairy cell leukemia
Pancytopenia, dry tap, tartrate-resistant acid phosphatase (TRAP) +, CD19, CD2OHairy cell leukemia
Prone for infection (Mycobacterium avium intracellulare). Treatment; Cladribine (2-CDA), which inhibits adenosine deaminase (ADA) and increases the level of toxic deoxyadenosineHairy cell leukemia
HTLV 1 infection, TAX gene (essential for viral replication)Adult T cell Leukemia / Lymphoma
Inactivates cell cycle inhibitor p16/INK4a and activates cyclin D1. TAX activates NF-kB (NF-kB is a transcription factor, is seen to help pro-survival/anti-apoptotic genes) . TAX gene inhibits p53Adult T cell Leukemia / Lymphoma
Skin infiltration (lesions), Hypercalcemia, ‘lytic’ bone lesions, CD4+Adult T cell Leukemia / Lymphoma
CD2, CD3, CD4, on skin biopsies, Pautrier microabscesses & cells with cerebriform nucleiMycosis Fungoides / Sézary syndrome
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WBC 4

Question Answer
Hypogranular granulocytes, pseudo Pelger-Huet cells (Bi-lobe Neutrophil)Myelodysplastic Syndrome
Myeloblasts LESS than 20% in bone marrow. Progresses to AMLMyelodysplastic Syndrome
Erythroid series: ringed sideroblasts, megaloblastoid cellsMyelodysplastic Syndrome
Myeloid series: pseudo Pelger-Huet cells; neutrophils with 2 nuclear lobesMyelodysplastic Syndrome
Megakaryocyte: pawn ball megakaryocytes (single nuclear lobes)Myelodysplastic Syndrome
Common feature in Chronic Myeloid Leukemia (CML), Polycythemia vera, Essential thrombocytosis & Primary myelofibrosisJAK 2
Total WBC: more than 100,000 cells/mm3. HUGE SPLEENChronic Myeloid Leukemia
@ the time of diagnosis myeloblastsare less than 5% (increase over 10% signifies onset of accelerated phase)Chronic Myeloid Leukemia
Philadelphia chromosome is associated with increased cell division and inhibition of apoptosisChronic Myeloid Leukemia & B-Cell Acute Lymphoblastic Leukemia
Translocation involving ABL gene on chromosome 9 and BCR gene on chromosome 22 activates multiple downstream pathways; RAS, JAK/STAT, AKTChronic Myeloid Leukemia
LAP Score is ____ Chronic Myeloid Leukemia & ____ in Leukemoid reactionLow & High
Low erythropoietin (EPO), increased red cell count, increased basophils, large plateletsPolycythemia Vera
Headache, dizziness, tinnitus, visual disturbances “ruddy” color of skin, especially facePolycythemia Vera
pruritus (release of histamine from mast cells/basophils), ‘aquagenic pruritus’Polycythemia Vera
PDGF and TGF-β released from neoplastic megakaryocytesPrimary Myelofibrosis
Normocytic normochromic anemia, nucleated rbc, ‘tear drop’ rbc (dacrocytes), early granulocytes, leukoerythroblastic reaction (leukoerythroblastosis)Primary Myelofibrosis
anemia, splenomegaly, fatigue, night sweats, hyperuricemia (gout), fibrosis of marrowPrimary Myelofibrosis
Erythromyalgia (intense burning or throbbing pain of hand and feet). Proliferation of plateletsEssential Thrombocytosis
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WBC 5

Question Answer
t(14;18) leads to overexpression of bcl-2Follicular B-Cell Lymphoma (NHL)
CD19, CD20, CD10. Transform to to “diffuse large B cell lymphoma".Follicular B-Cell Lymphoma (NHL)
Primary Effusion Lymphoma. HIV infectionDiffuse large B cell lymphoma
BCL-6Diffuse large B cell lymphoma
Rapidly enlarging lymph nodes at single nodal site or extra nodal site (GIT)Diffuse large B cell lymphoma
Burkitt Lymphoma, African (endemic) involves theJaw
Burkitt Lymphoma, Sporadic (non endemic) involves theileocecal region of the bowel (abdomen)
t(8; 14): myc gene on chr 8. Ig on chr 14Burkitt Lymphoma
“Starry sky” pattern on smear. B-cell expression of IgMBurkitt Lymphoma
t(11;l4) (cyclin D1& IgH)Mantle cell lymphoma
CD19, CD20, CD5+, CD23 NEGATIVEMantle cell lymphoma
Waldeyer ring (pharyngeal ring), and GIT (lymphmatoid polyp), Stage 4Mantle cell lymphoma
Extra nodal marginal zone MALToma; Stomach (H. Pylori), orbit, intestine, lung, thyroid (Hashimoto’s), salivary gland (Sjogren syndrome), bladder, CNS, chronic inflammation by autoimmuneMarginal zone Lymphomas
ALK gene that triggers JAK/STAT pathwayAnaplastic Large-cell Lymphoma (T-cell Lymphomas)
CD30 (Ki-1).Horseshoe nuclei (hallmark cells). Good prognosis.Anaplastic Large-cell Lymphoma (T-cell Lymphomas)
Lacunar/Reed-Sternberg (Owl Eye) cellHodgkin lymphoma
Nodular sclerosis (good prognosis)Hodgkin lymphoma
CD15, CD30 positive, involve lower cervical, supra clavicular and mediastinal lymph nodesHodgkin lymphoma
Pel-Epstein fever (fevers persist for days to weeks, followed by afebrile intervals and then recurrence of the fever)Hodgkin lymphoma
Association of ‘anemia of chronic disease’ (ACD) with normocytic normochromic anemia. Long term survivors of chemotherapy, and radiotherapy have an increased risk for second malignancy: AML in the setting of MDS, lung cancers, and breast cancersHodgkin lymphoma
Produces cytokines (IL-5, IL-10, 1L-13, & TGF-β)Reed-Sternberg Cells (Hodgkin lymphoma)
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WBC 6

Question Answer
Age for Multiple MyelomaElderly
Chronic, progressive & fatal malignant condition characterized by neoplastic proliferation of plasma cells which infiltrate the bone marrow.Multiple myeloma
Lytic bone lesions, low immunity, and AL amyloid deposits. Free L chains are known as Bence-Jones proteinsMultiple myeloma
IL- 6 is elevated, NF-kBMultiple myeloma
MIP 1α, NF-kB,RANK-L. These serve as osteoclast activating factorsMultiple myeloma
Fractures, hypercalcemia. Occupational exposure: radium, benzene, radiation. Earlier days there was an association with ThorostatMultiple myeloma
Rouleaux formation, flame cells, Mott cells, Russell bodies, Dutcher bodies. >30% plasma cellsMultiple myeloma
Increased bleeding time & ESR, proteinuria, presence of light chains (L), Bence-Jones proteinsMultiple myeloma
Streptococcus pneumoniae, Staphylococcus aureus & E.coli infectionsMultiple myeloma
‘M’ spike due to IgG, anemia, thrombocytopenia, abnormal plasma cells CD138+. Bone issues, infections, and renal failureMultiple myeloma
CRAB: elevated Calcium, Renal failure, Anemia, Bone lesionsMultiple myeloma
Isolated M spike ONLY. No lytic bone lesions, no hypercalcemia, no Bence Jones proteinMonoclonal Gammopathy of Uncertain Significance
Bone marrow less than 10% plasma cellsMonoclonal Gammopathy of Uncertain Significance
Lymphoplasmacytic lymphomaWaldenström macroglobinemia
Malignancy of B-cells characterized by monoclonal IgM. Hhyperviscosity due to increase monoclonal IgM (pentamer)Lymphoplasmacytic lymphoma
Tumor dissemination to lymph nodes, spleen and liver. infiltration of nerve roots and meninges may be seen later in the course of the diseaseLymphoplasmacytic lymphoma
Increase in mast cells (reactive), increase in lymphoid cells, rouleaux formationLymphoplasmacytic lymphoma
Cold agglutination’ due to IgM, Raynaud’s phenomenon may be seen. Monoclonal IgM have high affinity for myelinLymphoplasmacytic lymphoma
Eye hemorrhage, bleeding issues (coagulation factors), Raynauds’, Cold agglutinationLymphoplasmacytic lymphoma
Age for Lymphoplasmacytic lymphomaElderly
Birbeck granules (racquet-shaped), S-100 positive, CD1a positiveLangerhans cell Histiocytosis
Immature dendritric cellsLangerhans cell
Less than 2 years. Skin lesions, LN, Liver, spleen, bone destruction. Anemia, thrombocytopenia. BAD.Letterer-Siwe disease
Seen in children. Skull lesions, pituitary, exophthalmos. BADHand-Schuller-Christian
Seen in young adults. One Focal bone lesion, with a lot of eosinophils. GOODEosinophilic granuloma
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