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Hematology / Oncology > Blood Dyscrasia > Flashcards

Blood Dyscrasia Flashcards

1
Q

Acute Lymphocytic Leukemia (ALL)

A

MC malignancy in children under <15 years

Most responsive to therapy

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2
Q

ALL Clinical Features

A

CNS Involvement

Abnormal mucosal or cutaneous bleeding (due to thrombocytopenia)

Splenomegaly, hepatomegaly, lymphadenopathy

Bone and joint pain (invasion of the periosteum)

Anterior mediastinal mass (T-cell ALL)

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3
Q

ALL Diagnostics/Labs

A

WBC count is variable (from 1,000/mm3 to 100,000/mm3); significant numbers of blast cells (immature cells) in peripheral blood

Thrombocytopenia

Granulocytopenia

Electrolyte disturbances: hyperuricemia, hyperkalemia, hyperphosphatemia

Bone marrow biopsy:
Required for diagnosis

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4
Q

ALL Treatment

A

Tumor lysis syndrome:

Potential complication of chemotherapy seen in acute leukemias and high-grade Non-Hodgkin Lymphoma

Rapid cell death with release of intracellular contents

Causes hyperkalemia, hyperphosphatemia, hyperuricemia, and hypocalcemia with resultant sequelae related to each imbalance

Treat as a medical emergency

More than 75% of all children with ALL achieve complete remission

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5
Q

Acute myelogenous leukemia (AML)

A

Neoplasm of myelogenous progenitor cells

Occurs mostly in adults (accounts for 80% of adult acute leukemias)

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6
Q

AML Risk Factors

A

Radiation exposure

Myeloproliferative syndromes

Down syndrome

Chemotherapy (particularly alkylating agents: cyclophosphamide, cisplatin)

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7
Q

AML Clinical Features

A

Increased risk of bacterial infections (due to neutropenia)

Pneumonia, UTIs, cellulitis, pharyngitis, esophagitis

Associated with high morbidity and mortality; potentially life-threatening

Abnormal mucosal or cutaneous bleeding (due to thrombocytopenia)

CNS involvement: diffuse or local neurologic dysfunction (e.g., meningitis, seizures)

Skin nodules

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8
Q

AML Diagnostics

A

WBC count is variable (from 1,000/mm3 to 100,000/mm3); significant numbers of blast cells (immature cells) in peripheral blood

Bone marrow biopsy (required for diagnosis): Auer Rods

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9
Q

AML Auer Rods

A

Granules and eosinophilic rods inside malignant cells

Present in AML, but not in ALL

Particularly noted if it is the APL phenotype

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10
Q

AML Treatment

A

Tumor Lysis syndrome (like in ALL)

Aggressive, combo chemotherapy for several weeks at high doses until remission

More difficult to treat than ALL

Does not respond as well to chemotherapy

Bone marrow transplantation gives the best chance of remission or cure

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11
Q

Chronic Lymphocytic Leukemia (CLL)

A

Most common: most people with CLL are greater than 60 years of age

Leukemia occurring after age 50

Most common leukemia in the Western world

Monoclonal proliferation of B-type lymphocytes that are morphologically mature, but functionally defective

Generally regarded as the least aggressive type of leukemia

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12
Q

CLL Clinical Features

A

Usually asymptomatic at time of diagnosis; CLL may be discovered on a routine CBC (lymphocytosis)

Generalized painless LAD (nontender lymph nodes), splenomegaly

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13
Q

CLL Diagnostics

A

CBC – WBC of 50,000 to 200,000

Pancytopenia is common (anemia, thrombocytopenia, neutropenia)

Peripheral blood smear often diagnostic

Absolute lymphocytosis – almost all WBCs are mature, small lymphocytes

Presence of smudge cells – “fragile” leukemic cells that are broken when placed on glass slide

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14
Q

CLL Treatment

A

Chemotherapy:
Little effect on overall survival

Given for symptomatic relief and reduction of infection
Patients are often observed until symptoms develop

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15
Q

Chronic Myelogenous Leukemia (CML)

A

Associated with translocation t(9,22)

The fusion of the BCR gene on chromosome 22 with the ABL1 gene on chromosome results in the Philadelphia chromosome (present in > 90% of patients with CML)

Results in a constitutively active tyrosine kinase protein (targeted by imatinib (Gleevec))

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16
Q

CML Treatment

A

Treatment of CML = one of the great stories of modern medicine

After mechanism was identified, the oral TK inhibitor imatinib (Gleevec) was developed (one of the first targeted chemotherapies)

Drug targets the dysfunctional chimeric protein bcr-abl formed by the t(9,2) Philadelphia chromosome

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17
Q

Hodgkin Lymphoma (HL) General

A

Bimodal age distribution:
X1: 15 to 30 years of age
X2: > 50 years of age

Reed-Sternberg Cells

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18
Q

HL Clinical Features

A

Most common symptom is a painless lymphadenopathy

Constitutional (B) symptoms: weight loss, fever, night sweats, anorexia

Pruritus

Cough: secondary to mediastinal lymph node involvement

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19
Q

HL Diagnosis

A

Lymph node biopsy:
The presence of Reed-Sternberg cells is required to make the diagnosis

Neoplastic, large cell with two or more nuclei; looks like owl’s eyes

Usually B-cell phenotype

Presence of inflammatory cell infiltrates

Differentiates Hodgkin from non-Hodgkin lymphoma

20
Q

Non-Hodgkin Lymphoma (NHL)

A

Twice as common as Hodgkin disease

NHL tends to present with more advanced disease than those with HL

Etiology: unknown

21
Q

NHL Risk Factors

A

HIV/AIDS

Immunosuppression (e.g., organ-transplant recipient)

History of certain viral illnesses (e.g., EBV, HTLV-1)

History of Helicobacter pylori (risk of primary associated gastric lymphoma)

Autoimmune disease (e.g., Hashimoto’s thyroiditis or Sjögren syndrome (risk of mucosa-associated lymphoid tissue [MALT])

22
Q

NHL General

A

Classification:

More than 20 different subtypes of NHL

23
Q

NHL Indolent/Low Grade Subtype

Small lymphocytic lymphoma

A

Closely related CLL, more common in elderly patients

Indolent course

24
Q

NHL Indolent/Low Grade Subtype

Follicular, primarily small, cleaved-cell lymphoma

A

Most common form of NHL

Presents with painless, peripheral lymphadenopathy

Mean age of onset: 55 years of age

25
Q

NHL Intermediate Grade Subtype

Diffuse, large-cell lymphoma

A

Predominantly B-cell origin

Middle-aged and elderly patients

Locally invasive; presents as large extranodal mass

26
Q

NHL High Grade Subtype

Lymphoblastic lymphoma

A

T-cell lymphoma; more common in children

May progress to T-ALL

Aggressive with rapid dissemination, but may respond to combination chemotherapy

27
Q

NHL High Grade Subtype

Burkitt (small, non-cleaved cell) lymphoma

A

B-cell lymphoma; more common in children

African variety linked with EBV infection

Associated w/ specific translocation: t(8;14)

28
Q

NHL Miscellaneous Lymphomas

Mycosis fungoides

A

Presents with eczamatoid skin lesions that progress to generalized erythroderma

Cribiform shape of lymphocytes

29
Q

NHL Clinical Features

A

Lymphadenopathy:
Sometimes the only manifestation of the disease

Lymph nodes are usually painless, firm, and mobile

30
Q

NHL Diagnosis

A

Lymph node biopsy:

Definitive diagnosis

Any lymph node > 1 cm present for more than 4 weeks that cannot be attributed to infection should be biopsied

31
Q

Multiple Myeloma (MM)

A

Neoplastic proliferation of a single plasma cell line that produces monoclonal immunoglobulin

Incidence increases after age 50 years; twice as common in African-American patients than Caucasian patients

As the disease progresses, bone marrow elements are replaced by malignant plasma cells

32
Q

MM Clinical Features

A

Bone pain due to osteolytic lesions, fractures, and vertebral collapse

Anemia: Normocytic normochromic

Myeloma nephrosis: Immunoglobulin precipitation in renal tubules leads to tubular casts of Bence Jones protein

Up to 70% of patients die from infection: pulmonary or urinary tract are most common

Cord compression: Secondary to a plasmacytoma or fractured bone fragment

33
Q

MM Diagnostics - Labs

A

Hypercalcemia: due to bone destruction

Peripheral smear: RBCs seen in rouleaux formation

Urine: large amounts of free light chains called Bence Jones proteins

Leukopenia, thrombocytopenia, and anemia

Elevated creatinine = renal insufficiency

34
Q

MM Clinical Manifestations Pneumonic: CRAB

A 
C = Calcium issues) hypercalcemia)
R = Renal insufficiency
A = Anemia
B = Bone lesions and pain
35
Q

MM Treatment

A

Preferred treatment is autologous hematopoietic cell transplantation (HCT)

36
Q

Monoclonal gammaopathy of undetermined significance (MGUS)

A

Asymptomatic premalignant clonal plasma cell proliferation

Less than 10% plasma cells in bone marrow

Bence-Jones proteinuria < 1 gram/24 hours

Should also be NO end-organ damage (e.g., lytic bone lesions, anemia, hypercalcemia, etc.)

Almost like a minor form of MM

37
Q

Polycythemia vera (PV)

A

Malignant clonal proliferation of hematopoietic stem cells leading to excessive erythrocyte production

Mutations of the JAK2 tyrosine kinase are found in >90% of polycythemia vera cases

38
Q

PV Clinical features

A

Symptoms due to hyperviscosity: HA, dizziness, weakness, pruritus, visual impairment, dyspnea

Thrombotic phenomena

Bleeding

Splenomegaly, hepatomegaly

HTN

39
Q

PV Diagnostics

A

Rule out causes of secondary polycythemia (e.g., hypoxia, CO exposure)

CBC:
Elevated RBC count, H/H (usually >50%)
Thrombocytosis, leukocytosis may be present

Serum erythropoietin levels are reduced

Elevated vitamin B12 level

Hyperuricemia is common

Bone marrow biopsy confirms

40
Q

PV Differential Diagnosis

A
  1. Smoking: Lab evaluation shows increased Hct, RBC mass, EPO level, and carboxyhemoglobin
    No splenomegaly on PE
  2. Hypoxemia (secondary polycythemia): Living for prolonged periods at high altitudes
    Pulmonary fibrosis
    Congenital cardiac lesions
    Laboratory evaluation shows decreased arterial oxygen saturation
    No splenomegaly on PE
41
Q

PV Treatment

A

Repeated phlebotomy to lower the hematocrit

42
Q

Myelodysplatstic Syndrome

A

Class of acquired clonal blood disorders

Characterized by ineffective hematopoiesis, with apoptosis of myeloid precursors

Result is pancytopenia, despite normal or hypercellular bone marrow

43
Q

Myelodysplastic Syndrome Etiologies

A

Usually idiopathic

Exposure to radiation

Immunosuppressive agents

Certain toxins

44
Q

Myelodysplastic Syndrome Diagnosis

A

Bone marrow biopsy typically shows dysplastic marrow cells with blasts or ringed sideroblasts

45
Q

Myelodysplastic Syndrome Treatment

A

Pharmacologic therapies

Bone marrow transplantation is the only potential cure

Hematology / Oncology (4 decks)

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