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Hematology
FA complete review part 3.2 Pathology
| Question | Answer |
|---|---|
| What is the main defect of Porphyrias? | Conditions of defective heme synthesis that lead to the accumulation of heme precursors. |
| What enzymes are affected by lead poisoning? | Ferrochelatase and ALA dehydratase |
| What substrates are accumulated in Lead poisoning? | Protoporphyrin, and ALA |
| Inhibition of ALA dehydratase. Dx? | Lead poisoning |
| Inhibition of Ferrochelase. Dx? | Lead poisoning |
| Clinical symptoms of lead poisoning | - Microcytic anemia, GI and Kidney disease |
| What is a common way for a child to acquire lead poisoning? | Exposure to lead pain leading to mental deterioration. |
| What are common ways an adult gets Lead poisoning? | Batteries, and ammunition |
| What enzyme inhibition leads to Acute Intermittent Porphyria? | Porphobilinogen deaminase |
| What is the old name of Porphobilinogen deaminase? | Uroporphyrinogen I synthase |
| What substrates accumulated in AIP? | Porphobilinogen and ALA |
| What is the treatment of Acute Intermittent porphyria? | Hemin and glucose, which inhibit ALA synthase |
| Why is AIP is treated with hemin and glucose? | Inhibit ALA synthase |
| What is the mode of inheritance of Acute Intermittent porphyria? | Autosomal dominant mutation |
| What are the common symptoms of Acute Intermittent porphyria? | Painful abdomen Port wine - colored urine Polyneuropathy Psychological disturbances Precipitated by drugs, alcohol, and starvation |
| What type of CYP450 drugs may precipitate AIP? | Inducers |
| The 5 P's are a way to: | Recall the 5 main symptoms of AIP |
| What are the 5 P's of AIP? | Painful abdomen Port wine - colored urine Polyneuropathy Psychological disturbances Precipitated by CYP450 inducers, alcohol, and starvation |
| Inhibited Porphobilinogen deaminase. Dx? | Acute Intermittent porphyria |
| What condition is due to inhibition of Uroporphyrinogen I synthase? | Acute Intermittent porphyria |
| What enzyme is inhibited in Porphyria cutanea tarda? | Uroporphyrinogen decarboxylase |
| What substrate is accumulated in Porphyria cutanea tarda (PCT)? | Uroporphyrin |
| What is the result in urine due to Uroporphyrin accumulation in PCT? | Tea-colored urine |
| What are the presenting symptoms of PCT? | Blistering cutaneous photosensitivity and hyperpigmentation |
| What is the MC porphyria? | Porphyria cutanea tarda |
| What can exacerbate the development of Porphyria cutanea tarda? | Alcohol consumption |
| What virus is strongly associated with Porphyria cutanea tarda? | Hepatitis C virus |
| What enzyme is the rate-limiting step in heme production? | ALA synthase |
| What condition is associated with inhibition of ALA synthase? | Sideroblastic anemia |
| ALA dehydratase is inhibited in ________________________. | Lead poisining. |
| Which enzymes are inhibited in lead poisoning? | Ferrochelase and ALA dehydratase |
| What is the effect on ALA synthase by decreasing level of heme? | Increase ALA synthase activity |
| Increase heme ----> | Decrease ALA synthase activity |
| What is the mechanism of action of Iron (Fe+) poisoning? | Cell death due to peroxidation of membrane lipids. |
| What is the treatment of Iron poisoning? | 1. Chelation with IV deferoxamine, and Oral deferasirox 2. Dialysis |
| Why do kids often get iron poisoning? | Adult iron tablets may look like candy |
| What is the role of PT? | Test sufunio of common and extrinsic pathway |
| PT is associated with the ______________________ pathway. | Extrinsic |
| The extrinsic pathway is tested or monitored with ______. | PT |
| Extrinsic pathway ----- | PT |
| Defect on Extrinsic pathway lead to ----> | Increase PT |
| Play Tenis outside | PT -- Extrinsic |
| INR stands for: | International normalized ratio |
| What is the normal INR? | 1 |
| INR > 1 is considered ______ | Prolonged |
| What is monitored by PTT? | Function of common and intrinsic pathway |
| Which factors are monitored by PT? | 1, 2, 5, 7, and 10. |
| Which factors are monitored by PTT? | All factors except for 7 and 13 |
| Which factors are not monitored by PTT? | 7 and 13 |
| A defect in the Intrinsic pathway leads a _________ PTT. | Increase |
| Play Table Tennis Inside | PTT - Intrinsic |
| In hemophilias, which is prolonged, PT or PTT? | PTT |
| Hemophilia are an ____________________ pathway coagulation defect. | Intrinsic |
| Deficiency in coagulation factor 8? | Hemophilia A |
| Deficiency in coagulation factor 9? | Hemophilia B |
| Deficiency in coagulation factor 11? | Hemophilia C |
| Which hemophilias are X-linked recessive? | Hemophilia A and B |
| Which hemophilia is Autosomal recessive? | Hemophilia C |
| What is the main clinical symptoms or feature of hemophilia? | Hemarthrosis |
| Hemarthrosis, easy bruising, and bleeding after trauma or surgery. Dx? | Hemophilia |
| Which is the only hemophilia treated with desmopressin? | Hemophilia A |
| Desmopressin + factor VIII concentrate | Treatment for Hemophilia A |
| Mode of inheritance of Hemophilia B? | X-linked recessive |
| Which coagulation disorder shows prolonged PT and PTT? | Vitamin K deficiency |
| How are PT and PTT in vitamin K deficiency? | Both prolonged |
| General coagulation defect with normal bleeding time. Dx? | Vitamin K deficiency |
| Mechanism of pathogenesis of Vitamin K deficiency | Decreased activity of factors 2, 7, 9, 10 and protein C, protein S. |
| What factors are activated by Vitamin K? | 2, 7, 9, 10 and proteins C and S. |
| Coagulation disorders are: | 1. Hemophilia A, B, C 2. Vitamin K deficiency |
| Coagulation disorders show prolongation in: | PT and PTT |
| List of Platelet disorders: | 1. Bernard-Soulier syndrome 2. Glanzmann thrombasthenia 3. Hemolytic-uremic syndrome (HUS) 4. Immune thrombocytopenia 5. Thrombotic thrombocytopenia purpura |
| Which measured is prolonged in all platelet disorders? | BT |
| What are the two main measures affected in platelet disorders? | PC (platelet count) and BT (bleeding time) |
| Which platelet disorders always have decreased PC? | HUS, ITP & TTP |
| Which platelet disorder may be seen with a decreased PC? | Bernard-Soulier syndrome |
| Defect in platelet plug formation. Dx? | Bernard-Soulier syndrome |
| Pathogenesis of Bernard-Soulier syndrome? | Decreased GpIb ---> defect in platelet-to-vWF adhesion |
| What protein or receptor is affected in Bernard-Soulier syndrome? | GpIb |
| What is the error or defect in adhesion in Bernard-Soulier syndrome? | Platelet-to-vWF |
| Abnormal ristocetin test that does not correct with mixing studies. | Bernard-Soulier syndrome |
| Which platelet disorder is seen with decreased GpIb activity? | Bernard-Soulier syndrome |
| Defect in platelet integrin GpIIb/IIIa. Dx? | Glanzmann thrombasthenia |
| What integrin (protein) is affected in Glanzmann thrombasthenia? | GpIIb/IIIa |
| Glanzmann thrombasthenia shows a defect in --> | Platelet-to-platelet adhesion |
| What is seen in the labs of Glanzmann thrombasthenia? | 1. Normal PC 2. Increased BP 3. Blood smear show no platelet clumping |
| What is seen in the blood smear of Glanzmann thrombasthenia patient? | No platelet clumping |
| Defective GpIIb/IIIa. Dx? | Glanzmann thrombasthenia |
| What is the triad of HUS? | 1. Thrombocytopenia 2. Microangiopathic hemolytic anemia 3. Acute renal failure |
| PC in HUS is _________________. | Decreased |
| What is the treatment for HUS? | Plasmapheresis |
| What is the main accompanying symptom in children with HUS? | Diarrhea |
| MC Causative agent of HUS in children | Shiga-like toxin of EHEC |
| EHEC toxing causative of HUS | Shiga-like toxin |
| What is different in adult presentation of HUS, compared to a child? | Adult HUS does not present with diarrhea, and EHEC infection is not required |
| Anti-GpIIb/IIIa antibodies. Dx? | Immune thrombocytopenia |
| Anti-GpIIb/IIIa antibodies cause --> | Splenic macrophage consumption of platelet-antibody complex |
| What are lab findings fo Immune thrombocytopenia? | Increased megakaryocytes on bone marrow biopsy |
| Treatment of Immune thrombocytopenia (ITP) | -Steroids, IVIG - Rituximab or splenectomy for refractory ITP |
| Rituximab is used to treat which platelet disorder? | Immune thrombocytopenia |
| Inhibition or deficiency of ADAMTS 13. Dx? | Thrombotic thrombocytopenic purpura (TTP) |
| Another name for ADAMTS13 | vWF metalloprotease |
| What is the result of the deficiency of ADAMTS13? | Decreased degradation of vWF multimers |
| Pathogenesis of TTP: | Increased large VWF multimers --> increased platelet adhesion leading to increased platelet aggregation and thrombosis. |
| Important lab findings of TTP: | Schistocytes, elevated LDH, normal coagulation parameters. |
| What is the pentad of symptoms of TTP? | Fever Microangiopathic hemolytic Anemia Thrombocytopenia Renal failure Neurological symptoms |
| FAT RN | Mnemonic for Pentad of symptoms in TTP |
| What does FAT RN stand for? | Fever microangiopathic hemolytic Anemia Thrombocytopenia Renal failure Neurological symptoms |
| What are two common mixed platelet and coagulation disorders? | 1. von Willebrand disease 2. Disseminated intravascular coagulation |
| In von Willebrand disease which is the only measure that is always prolonged? | Bleeding time (BT) |
| What is the Intrinsic coagulation defect of vWD? | Decreased vWF leading to increased PTT |
| What is the platelet plug formation defect in vWD? | Decreased vWF leading to defect in platelet-to-vWF adhesion |
| What is the MC inherited bleeding disorders? | von Willebrand disease |
| What is the treatment of von Willebrand disease? | Desmopressin |
| Why is desmopressin used to treat VWD? | Releases vWF stored in endothelium |
| Medication the releases vWF stored in endothelium. | Desmopressin |
| What is the role of vWF? | Act to carry/protect factor 8 |
| In DIC, which parameters are elevated or increased? | BT, PT, and PTT |
| Which condition/pathology is seen with decreased PC, and elevated BT, PT, and PTT? | Disseminated intravascular coagulation (DIC) |
| Widespread activation of clotting leading to deficiency in clotting factors resulting in a bleeding state. | Mechanism of action DIC |
| What are the most common causes of DIC? | - Sepsis (gram (-)) - Trauma - Obstetric complications - Acute Pancreatitis - Malignancy - Nephrotic syndrome - Transfusion |
| What are the labs in DIC? | - Schistocytes - Increased fibrin degradation products (D-dimers) - Decreased fibrinogen - Decreased factor V and VIII |
| Increased level of D-dimers, commonly seen with: | Disseminated intravascular coagulation (DIC) |
| Which is the parameter decreased in DIC? | PC (platelet count) |
| List of Hereditary thrombosis syndromes leading to hypercoagulability: | 1. Antithrombin deficiency 2. Factor V Leiden 3. Protein C or S deficiency 4. Prothrombin gene mutation |
| What is the effect seen with inherited antithrombin deficiency? | Diminishes the increase in PTT following heparin administration |
| What is the pathogenesis of acquired antithrombin deficiency? | Renal failure/nephrotic syndrome ---> antithrombin loss in urine which causes a decreased inhibition of factors IIa and Xa |
| What is the mutation seen in Factor V Leiden? | G --> A DNA point mutation ---> Arg506Gln neate the cleavage site |
| Factor V resistant to degradation by activated protein C. Dx? | Factor V Leiden |
| What is the MCC of hypercoagulability syndrome of Caucasians? | Factor V Leiden |
| What are common complications of Factor V Leiden? | DVT, cerebral vein thromboses, and recurrent pregnancy loss |
| Decreased ability to inactivate factors Va and VIIIa. Dx? | Protein C and S syndrome |
| Protein C and/S deficiency increases the risk of: | Thrombotic skin necrosis with hemorrhage after administration of warfarin |
| Which anticoagulant is associated with skin necrosis in patients with Protein C deficiency? | Warfarin |
| Protein C ________________ coagulation | Cancels |
| Protein S __________________ coagulation. | Stops |
| What is the mutation of Prothrombin gene? | Mutation in 3' untranslated region |
| What is the end result of Prothrombin gene mutation? | Increased plasma levels and venous clots |
| Which conditions are treated with Packed RBCs as blood transfusion component? | Acute blood loss, and severe anemia |
| What is the effect of transfusing packed RBCs? | Increase Hb and Oxygen carrying capacity |
| Clinical use of platelet transfusion: | Stop significant bleeding |
| Which conditions use FFP/Prothrombin complex concentrate in transfusion therapy? | DIC, cirrhosis, immediate anticoagulation reversal |
| Immediate anticoagulation reversal is accomplished with: | Fresh Frozen Plasma (FFP) |
| What is the main effect of FFP? | Increase coagulation factor levels |
| What is FFP composed of? | All coagulation factors and plasma proteins |
| What factors are contained in PCC (Prothrombin complex concentrate)? | Factors II, VII, IX, and X as well as protein C and S |
| What does cryoprecipitate contains? | Fibrinogen, factor VIII, factor XIII, vWF, and fibronectin |
| What blood therapy component is used in causes of coagulation factor deficiency of fibrinogen and factor VIII? | Cryoprecipitate |
| What are possible blood transfusion risks? | 1. Infection transmission (low) 2. Transfusion reactions 3. Iron overload (secondary hemochromatosis) 4. Hypocalcemia 5. Hyperkalemia |
| How does blood transfusion therapy may lead to Hypocalcemia? | Citrate is a Ca2+ chelator |
| How does blood transfusions cause risk of hyperkalemia? | RBCs may lyse old blood units |
| What is Leukemia? | Lymphoid or myeloid neoplasm with widespread involvement of bone marrow. |
| Where do tumor cells of leukemia found? | Peripheral blood |
| What is a Lymphoma? | Discrete tumor mass arising form lymph nodes |
| Small, diffuse tumor mass originated from lymph nodes. Dx? | Lymphoma |
| What are the two types of Lymphomas? | - Hodgkin and, - Non-Hodgkin |
| Shared symptoms of Hodgkin and non-Hodgkin lymphomas? | Constitutional "B" symptoms |
| What are the constitutional "B" symptoms? | Low-grade fever Night sweats Weight loss |
| Localized, single group of nodes; contiguous spread. Dx? | Hodgkin lymphoma |
| Contagious spread, Hodgkins or non-Hodgkin? | Hodgkin lymphoma |
| Which has better prognosis, Hodgkin or non-Hodgkin? | Hodgkin lymphoma |
| Which type of lymphoma is characterized by Reed-Sternberg cells? | Hodgkin lymphoma |
| Which type of lymphoma presents with bimodal distribution? | Hodgkin lymphoma |
| All Hodgkin lymphoma subtypes are most common in men, except for _________________________________. | Nodular Sclerosing type |
| Which lymphoma is strongly associated with EBV? | Hodgkin lymphoma |
| Occur in young adulthood and >. 55 years old. | Bimodal distribution of Hodgkin lymphoma |
| What virus is associated with development of Hodgkin lymphoma? | EBV |
| Which lymphoma is localized in multiple lymph nodes? | Non-Hodgkin lymphoma |
| Non-contiguous spread + Extranodal involvement. Dx? | Non-Hodgkin lymphoma |
| Histology of Non-Hodgkin lymphomas? | Majority involve B cells; a few are of T-cell lineage. |
| Lymphoma that can occur in children and adults? | Non-Hodgkin lymphoma |
| Associated virus of Non-Hodgkin lymphomas? | HIV |
| Which type of lymphoma is associated with HIV and autoimmune diseases? | Non-Hodgkin lymphoma |
| What is the key histological feature or finding of Hodgkin lymphomas? | Reed-Sternberg cells |
| Description of Reed-Sternberg cells | Distinctive tumor giant cells; Binucleate or bilobed with the 2 halves as mirror images |
| RS cells are positive for: | CD15+ and CD30+ |
| CD15+ and CD30+ cells. Dx? | Hodgkin lymphoma |
| 2 owl eyes x 15 = 30 | Mnemonic to remember that RS cells are binucleate with mirror images of nucleus, and are positive for CD15 and 30. |
| What is the MC subtype of Hodgkin lymphoma? | Nodular sclerosis |
| Which Hodgkin lymphoma subtype has the best prognosis? | Lymphocyte rich |
| What are some features of Mixed cellularity Hodgkin lymphoma? | Eosinophilia, seen in immunocompromised patients |
| Which two subtypes of Hodgkin lymphoma are commonly seen in immunocompromised patients? | Lymphocyte depleted and Mixed cellularity |
| Which genre is most affected, overall, by Hodgkin lymphoma? | Male |
| Which Hodgkin lymphoma type is the only one that affects women more than men? | Nodular Sclerosing type |
| List of B-cell Non-Hodgkin lymphomas: | 1. Burkitt lymphoma 2. Diffuse large B-cell lymphoma (DLBCL) 3. Follicular lymphoma 4. Mantle cell lymphoma 5. Marginal zone lymphoma 6. Primary CNS lymphoma |
| Burkitt lymphoma occurs in: | Adolescents and young adults |
| t(8;14). Dx? | Burkitt lymphoma |
| Translocation of c-myc (8) and heavy-chain Ig (14). Dx? | Burkitt lymphoma |
| Virus associated with Burkitt lymphoma? | EBV |
| Histological description of Burkitt lymphoma: | "Starry sky" appearance, sheets of lymphocytes with interspersed "tingible body" macrophages |
| "Starry sky" appearance. Describes which Non-Hodgkin lymphoma? | Burkitt lymphoma |
| Clinical presentation of Burkitt lymphoma | Jaw lesion in endemic form in Africa |
| What is the sporadic form of Burkitt lymphoma presentation? | Pelvis or abdomen lesion |
| Jaw mass in African adolescent. Dx? | Burkitt lymphoma |
| Alterations in Bcl-2, Bcl-6. Dx? | Diffuse large B-cell lymphoma |
| What is the most common type of non-Hodgkin lymphoma in adults? | Diffuse large B-cell lymphoma |
| What population is often affected by DLBCL? | Usually older adults, but 20% in children |
| t(14;18). Dx? | Follicular lymphoma |
| Which B-cell non-Hodgkin lymphomas affect children? | 1. Burkitt lymphoma (MC) 2. Diffuse large B-cell lymphoma (20%) |
| Translocation of heavy-chain Ig (14) and BCL-2 (18). Dx? | Follicular lymphoma |
| What is the usual clinical presentation of Follicular lymphoma? | Painless "waxing and waning" lymphadenopathy |
| Which NH lymphoma presents with Indolent course of symptoms? | Follicular lymphoma |
| What is the purpose of Bcl-2? | Inhibition of apoptosis |
| Which NH lymphoma mostly occurs to adult males? | Mantle cell lymphoma |
| Associated translocation of Mantle cell lymphoma? | t(11;14) |
| Translocation of cyclin D1 (11) and heavy-chain Ig (14). Dx? | Mantle cell lymphoma |
| CD5+ NH-lymphoma. | Mantle cell lymphoma |
| Mantle cell lymphoma is (+) for which CD marker? | CD5+ |
| t(11;18). Dx? | Marginal zone lymphoma |
| What are important associations of Marginal zone lymphoma? | 1. Sjogren syndrome 2. Chronic gastritis (MALT lymphoma) |
| Which Non-Hodgkin lymphoma is associated with chronic inflammation? | Marginal zone lymphoma |
| Marginal zone lymphoma associated translocation. | t(11;18) |
| What is the most common association of Primary CNS lymphoma? | HIV/AIDS |
| What virus is involved in the pathogenesis of Primary CNS lymphoma? | EBV |
| Which NH lymphoma is considered an AIDS-defining illness? | Primary CNS lymphoma |
| What is the description of Primary CNS lymphoma on MRI imaging? | Ring-enhancing lesion in immunocompromised |
| Which are the two Non-Hodgkin lymphomas of mature T-cell origin? | 1. Adult T-cell lymphoma 2. Mycosis fungoides/Sezary syndrome |
| What is the cause of Adult T-cell lymphoma? | HTLV |
| What dangerous activity is associated with HLTV? | IV drug abuse |
| What is the most common clinical feature of Adult T-cell lymphoma? | Cutaneous lesions |
| Which are the most common countries with Adult T-cell lymphomas? | Japan, West Africa, and the Caribbean |
| What are clinical symptoms/labs of Adult T-cell lymphoma? | Lytic bone lesions and Hypercalcemia |
| What is Mycosis fungoides? | Skin patches/plaques, characterized by atypical CD4+ cells with "cerebriform" nuclei and intraepidermal neoplastic cell aggregates. |
| What are the Pautrier microabscesses? | Intraepidermal neoplastic aggregates see in in Mycosis fungoides. |
| Another name for T-cell leukemia? | Sezary syndrome |
| What is a possible severe complication of Mycosis fungoides? | Sezary syndrome |
| Multiple myeloma is: | Monoclonal plasma cell cancer |
| Multiple myeloma arise from: | Marrow and produces large amounts of IgG or IgA |
| What is the most common primary tumor arising from within the bone? | Multiple myeloma |
| Important associations of Multiple myeloma: | 1. Increased susceptibility to infection 2. Primary amyloidosis (AL) 3. Punched-out lytic bone lesions on x-ray 4. M spike on serum protein electrophoresis 5. Ig light chains in urine (Bence Jones protein) 6. Rouleaux formation of RBCs |
| What type of lesions are seen MM? | Punched-out lytic bone lesions on x-ray |
| M spike on serum protein electrophoresis. Dx? | Multiple myeloma |
| What is the common name of the Ig light chains in urine in a patient with Multiple myeloma? | Bence Jones proteins |
| PBS shows staked like poker chips RBCs. Dx? | Multiple myeloma |
| What are the immunoglobulins produced in Multiple myeloma? | IgG (%) and IgA (25%) |
| What are the histological findings of Multiple myeloma? | Numerous plasma cells with "clock-face" chromatin and intracytoplasmic inclusions containing immunoglobulin |
| CRAB is a mnemonic used in ____________ ________________. | Multiple myeloma |
| What does "CRAB" stand for? | - hyperCalcemia - Renal involvement - Anemia - Bone lytic lesions/Back pain |
| Monoclonal M protein spike. Dx? | Multiple myeloma |
| M spike = IgM. Dx? | Waldenstrom macroglobulinemia |
| What is Waldenstrom macroglobulinemia? | Hyperviscosity syndrome with no CRAB findings. M spike is caused by IgM. |
| What condition of IgM hypersecretion is seen with Raynaud phenomenon? | Waldenstrom macroglobulinemia |
| What does MGUS stand for? | Monoclonal gammopathy of undetermined significance |
| Monoclonal expansion of plasma cells, asymptomatic, may lead to multiple myeloma. | Monoclonal gammopathy of undetermined significance |
| Which plasma cell neoplastic pathology is seen with "CRAB" findings? | Multiple myeloma |
| A patient with ________________ develop MM at a rate of 1-2% per year. | MGUS |
| What are Myelodysplastic syndromes? | Stem-cell disorders involving ineffective hematopoiesis leading to defects in cell maturation of non lymphoid lineages. |
| What are the common causes of Myelodysplastic syndromes? | 1. de Novo mutations 2. Environmental exposure to radiation, benzene, chemotherapy |
| What is a severe complication of untreated/uncontrolled Myelodysplastic syndromes? | Transformation to AML |
| What is Pseudo-Pelger-Huet anomaly? | Neutrophils with bilobed nuclei; typically seen after chemotherapy. |
| Neutrophil shows a bilobed or "duet" nucleus appearance. Dx? | Pseudo-Pelger-Huet anomaly |
| What activity often leads to development of Pseudo-Pelger-Huet anomaly? | Chemotherapy |
| Unregulated growth and differentiation of WBCs in bone marrow. Dx? | Leukemias |
| What are the types of Lymphocytic leukemias? | 1. Acute lymphoblastic leukemia/lymphoma 2. Chronic lymphocytic leukemia/Small lymphocytic lymphoma 3. Hairy cell leukemia |
| What is the most common leukemia in children? | ALL |
| How is T-cell ALL presented? | Mediastinal mass |
| SVC-like syndrome leukemia. Dx? | ALL |
| What trisomy is strongly associated with ALL? | Down syndrome |
| TdT (+) | ALL of T-cell origin |
| What is the marker for ALL of pre-B cell origin? | CD10+ |
| What is the leukemia most responsive of therapy? | ALL |
| What is the most probable places that ALL may spread? | CNS and testes |
| t(12;21). Dx? | ALL |
| Marker of pre-B cells in ALL | CD10+ |
| Marker of pre-T cells and pre-B cells ALL | TdT+ |
| What is the most common adult leukemia? | CLL/SLL |
| CLL is positive to: | CD20+, CD23+, CD5+ B cell neoplasm |
| PBS shows smudge cells. Dx? | CLL/SLL |
| CLL is associated with ______________ _____________ anemia. | Autoimmune hemolytic |
| What is Richter transformation? | CLL/SLL transformation into aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). |
| CLL may transform into ____________________. | Diffuse large B-cell lymphoma |
| What is a mature B-cell tumor with cells have filamentous, hair-like projections? | Hairy cell leukemia |
| Dry tap on aspiration. Leukemia? | Hairy cell leukemia |
| Hairy cell leukemia often presents with: | Pancytopenia and massive splenomegaly. |
| Massive splenomegaly. Dx? | Hairy cell leukemia |
| Hairy cell leukemia stains (+) for: | TRAP |
| What is the treatment of Hairy cell leukemia? | Cladribine and Pentostatin |
| Leukemia treated with Cladribine. Dx? | Hairy cell leukemia |
| Important histological findings of Acute myelogenous leukemia (AML): | - Auer rods - Myeloperoxidase (+) cytoplasmic inclusions seen with APL |
| What was the old denomination of APL? | M3 AML |
| Significantly elevated circulating myeloblasts on peripheral smear. Dx? | AML |
| What is the average age for developing AML? | 65 years |
| What associated risk factors of AML? | - Alkylating chemotherapy - Radiation - Myeloproliferative disorders - Down syndrome |
| What is the translocation of AML? | t(15;17) |
| What is the treatment for APL t(15;17)? | all-trans retinoic acid (vitamin A) |
| What is the purpose of treating APL with all-trans retinoic acid? | Induce differentiation of promyelocytes |
| What is a common presentation of APL? | DIC |
| What is the age spectrum of CML? | 45-85 years old. |
| Auer rod (+) leukemia? | AML |
| What is the Philadelphia chromosome? | t(9;22) |
| BCR-ABL. Dx? | CML |
| What are clinical and histological features of CML? | Dysregulated production of mature and maturing granulocytes and splenomegaly. |
| CML goes into a "blast crisis" leading to --> | Transform of AML or ALL |
| Very low LAP as a result of low activity in malignant neutrophils. Dx? | CML |
| Increased LAP is seen with _________ _______________. | Leukemoid reaction |
| What kind of monoclonal antibodies are used in CML? | bcr-abl tyrosine kinase inhibitors |
| What are common bcr-abl tyrosine inhibitors? | Imatinib and Dasatinib |
| Imatinib is used to treat? | CML |
| What are common myeloproliferative disorders? | 1. Polycythemia vera 2. Essential thrombocytopenia 3. Myelofibrosis |
| What are myeloproliferative disorders? | Malignant hematopoietic neoplasms with varying impacts on WBCs and myeloid cell lines |
| Associated with V617F JAK2 mutation. | Chronic myeloproliferative disorders |
| Primary polycythemia. Disorders of RBCs. Dx? | Polycythemia vera |
| Intense itching after a hot shower. Dx? | Polycythemia vera |
| What is the classic symptom of Polycythemia vera? | Erythromelalgia |
| What is erythromelalgia? | Severe, burning pain and red-blue coloriation |
| What causes erythromelalgia in Polycythemia vera? | Episodic blood clots in vessels of the extremities. |
| Primary polycythemia is seen with ___________ EPO. | Decreased |
| What is the treatment of Polycythemia vera? | Phlebotomy, hydroxyurea, ruxolitinib |
| What class of drug is Ruxolitinib? | JAK1/2 inhibitor |
| Massive proliferation of megakaryocytes and platelets. Dx? | Essential thrombocytopenia |
| What are the most common symptoms of Essential thrombocytopenia? | Bleeding and thrombosis |
| What is Myelofibrosis? | Obliteration of bone marrow with fibrosis due to increased fibroblast activity. |
| What are the characteristic RBCs in Myelofibrosis? | "Teardrop" RBCs |
| JAK2 mutations associated conditions? | Polycythemia vera, Essential thrombocytopenia, and Myelofibrosis. |
| What are the classifications of Polycythemia? | Relative, Appropriate absolute, Inappropriate absolute, and Polycythemia vera. |
| What are causes of Relative Polycythemia? | Dehydration and burns |
| What conditions lead to Appropriate absolute polycythemia? | Lung disease, congenital heart disease, and high altitude. |
| What is the primary disturbance in Relative Polycythemia? | Decreased plasma volume |
| Which category of polycythemia is due primarily to a decrease in Oxygen saturation? | Appropriate absolute polycythemia |
| What is the primary disturbance of Appropriate absolute polycythemia? | Elevated EPO levels |
| What is a typical example of Inappropriate absolute polycythemia due to elevated exogenous EPO levels? | Athlete abuse ("blood doping") |
| An athlete that is accused of "blood doping" . Polycythemia type? | Inappropriate absolute polycythemia |
| What are some causes of Inappropriate EPO secretion? | Malignancy (RCC, HCC) |
| What are the two MC malignancies leading to elevated EPO levels? | RCC and HCC |
| Which condition (polycythemia) is associated with severely elevated RBC mass? | Polycythemia vera |
| What are associations of Polycythemia vera? | EPO decreased in PCV due to negative feedback suppression renal EPO production. |
| Which lymphoma is associated with BCL-2 activation? | Follicular lymphoma |
| APL is also known as: | M3 type of AML |
| Responds to all-trans retinoic acid | APL |
| Lymphoma associated with cyclin D1 activation | Mantle cell lymphoma |
| c-myc activation. | Burkitt lymphoma |
| BCR-ABL hybrid. | CML (most common), and ALL |
| Collective group of proliferative disorders of dendritic (Langerhans) cells? | Langerhans cell histiocytosis |
| What are presenting symptoms of Langerhans cell histiocytosis? | - Lytic bone lesions - Skin rash or, - Recurrent otitis media with a mass involving the mastoid bone |
| In Langerhans cell histiocytosis, otitis media presents with a mass involving which bone? | Mastoid bone |
| What is the deficit in the cells in Langerhans cell histiocytosis? | Functionally immature and do not effectively stimulate primary T cells via antigen presentation. |
| What is expressed in cells of Langerhans cell histiocytosis? | - S-100 (+) - CD1a (+) |
| What does (+) S-100 indicate? | Mesodermal origin |
| What is the characteristic histological finding of Langerhans cell histiocytosis? | Birbeck granules |
| Which condition is associated or diagnosed by the presence of Birbeck granules? | Langerhans cell histiocytosis |
| What are Birbeck granules? | Histological findings of LCH shaped as "tennis rackets" or rod-shaped on EM. |
| "Tennis racket" rods. Dx? | Langerhans cell histiocytosis |
| What is Tumor Lysis syndrome? | Oncologic emergency triggered by massive tumor cell lysis, most often in lymphomas/leukemias |
| Which malignancy conditions are the ones that most often present with Tumor Lysis syndrome? | Lymphomas and Leukemias |
| What is the preventive treatment for Tumor Lysis syndrome? | 1. Aggressive hydration 2. Allopurinol 3. Rasburicase |
| What electrolyte abnormalities are associated with Tumor Lysis syndrome (TLS)? | Hyperkalemia Hyperphosphatemia Hypocalcemia Hyperuricemia |
| What is the complication of Hyperuricemia in Tumor lysis syndrome? | Acute kidney injury |
| Reason for hyperuricemia in Tumor lysis syndrome | Increase nucleic acid breakdown |
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rakomi
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