WBC, Lymph Nodes, Spleen and Thymus Flashcards by Jamaira Belmes

Decreased number of circulating leukocytes
MC involves neutrophils (NEUTROPENIA)

Deficiency of lymphocytes (LYMPHOPENIA) - less common; commonly seen in advanced HIV and other diseases

LEUKOPENIA

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Clinically significant neutropenia <500/mm3

AGRANULOCYTOSIS

-high susceptibility to infections (Candida and Aspergillus)

-DRUG TOXICITY - MCC

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Enlargement of a lymph node as immune response to foreign antigens; histology usually nonspecific; depends on duration of disease and type of offending agent

Reactive Lymphadenitis

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7/F with OSA secondary to CHRONIC HYPERTROPHIC TONSILS, GR 3; underwent BILATERAL TONSILLECTOMY and ADENOIDECTOMY

Biopsy of the adenoids: increase in the number of germinal centers (secondary follicles) per unit area

DIAGNOSIS

Follicular Hyperplasia

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27/F developed FEVER and LYMPHADENOPATHY.

Serology: heterophil antibodies (+)
Diagnosis: Infectious Mononucleosis

Lymph node biopsy: expansion of the paracortical zones imparting a mottled appearance

Diagnosis

Paracortical Hyperplasia

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59/F, known case of breast cancer, underwent SENTINEL LYMPH NODE BIOPSY

Lymph node biopsy: (-) evidence of metastasis; (+) distention of lymphatic sinusoids by histiocytes

Sinusoidal Hyperplasia/Sinus Histiocytes

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Neoplastic proliferation of white cells wherein the neoplastic white cells populate the peripheral blood and bone marrow which leads to peripheral blood cytopenias.

Clinical manifestations depend on the cell line/s affected

Leukemia

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Neoplastic proliferation of white cells that presents as DISCRETE TISSUE MASSES

Lymphoma

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4/F presents w/ PALLOR, RECURRENT URTI and EPISTAXIS

PE: increased liver span and obliterated Traube space

CBC: 35% blast count w/ lymphocytic background

BM: hypercellular marrow w/ diffuse infiltration of blast cells

Flow Cytometry: 89% bone marrow blasts

Acute Lymphoblastic Leukemia (ALL) (precursor B-cell ALL)

Myeloid markers: MPO, CD117, CD33 (-)
Lymphoid markers: Tdt (+)
B-cell lineage markers: CD79a, CD19 (+)
T-cell lineage markers: CD3, CD7 (-)

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12/M presents w/ MEDIASTINAL MASS

CT: multiple mass in the abdomen and mediastinum

CBC: 40% blast count w/ lymphocytic background

BM: hypercellular marrow w/ diffuse infiltration of blast cells

Flow Cytometry: 78% bone marrow blasts

Precursor T-cell ALL

Myeloid markers: MPO, CD117, CD33 (-)
Lymphoid markers: Tdt (+)
B-cell lineage markers: CD79a, CD19 (-)
T-cell lineage markers: CD3, CD7 (+)

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40/M presents w/ BLEEDING

CBC: thrombocytopenia, 15% blasts count, w/ 40% nucleated WBCs w/ Auer rods obscuring the supposed perinuclear clearing (Golgi zone) of a promyelocyte

Coags: elevated PT/PTT

FISH: (+) t(15;17) PML-RARA

Acute Promyelocyte Leukemia (WHO Class I)

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36/M, known case of HODGKIN LYMPHOMA stage IIIB underwent chemotherapy w/ alkylating agents developed recurrent epistaxis

PE: increased liver span and obliterated Traube space

CBC: anemia, thrombocytopenia, leukocytosis (200 000/mm3) w/ 40% blast count

BM: hypercellular marrow w/ diffuse infiltration of blast cells

Flow cytometry: 92% BM blasts

Acute Myeloid Leukemia (AML
Therapy Related AML (WHO Class II)

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68/M, w/ MULTIPLE LYMPHADENOPATHY

CBC: 300 000/mm3 w/ 95% lymphocytes

PBS: lymphocytosis w/ SMUDGE CELLS

BM: hypercellular marrow w/ nodular lymphoid aggregates

LN Biopsy: diffuse but vaguely nodular infiltration of small lymphocytes w/ PROLIFERATION CENTERS (pseudofollicular pattern)

DIAGNOSIS

Chronic Lymphocytic Leukemia (CLL)

IHC: CD3 (-), CD20, CD5, CD23(+)

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45/F apparently asymptomatic

CBC: 300 000/mm3 w/ neutrophilia, left shift (myelocytes and metamyelocytes) and 2% blast count

LAP scores: LOW

Diagnosis

Chronic Myelogenous Leukemia (CML)

FISH studies: (+) BCR-ABL1 t(9:22)

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Hodgkin Lymphoma

Lymph node involvement: SINGLE, AXIAL group of nodes (cervical, mediastinal. para-aortic)
Spread: Contiguous
Mesenteric nodes and Waldeyer ring involvement: RARE
Extranodal presentation: RARE

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Non-Hodgkin Lyphoma

Lymph node involvement: MULTIPLE, PERIPHERAL NODES
Spread: Non-contiguous
Mesenteric nodes and Waldeyer ring involvement: COMMON
Extranodal presentation: COMMON

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23/F, Filipino w/ ling standing SOLITARY LEFT LATERAL MASS w/ fever and night sweats
PPD (+)

LN Biopsy: mixed infiltrate of inflammatory cells w/ large cells w/ multiple nuclei, each of which has a nucleolus (Owl eye) nuclei; (-) fibrous nodules

Mixed Cellularity HL.

IHC: CD15(+) and CD30 (+)

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23/F, Filipino w/ ling standing SOLITARY LEFT LATERAL MASS w/ fever and night sweats
PPD (+)

LN Biopsy: (+) fibrous nodules, (+) scattered cells, (-) necrosis

Nodular Sclerosis HL (Grade 1)

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23/F, Filipino w/ ling standing SOLITARY LEFT LATERAL MASS w/ fever and night sweats
PPD (+)

LN Biopsy: SCANt lymphocytic infiltrate w/ predominance o large pleomorphic cells; some of which look like RS cells

Lymphocyte-depleted HL

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Processes that distort the marrow architecture, such as deposits of metastatic cancer or granulomatous disorders, can cause the abnormal release of immature precursors into the peripheral blood. This finding is referred to as

Leukoerythroblastosis

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MYELOID

erythroid (RBCs)
granulocytic - neutrophils, eosinophils, basophils
monocytic
megakaryocytic - platelets

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LYMPHOID

lymphocytes (B and T cells)
plasma cells (from B cells)
natural killer (NK) cells

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MCC in children

ALL

Early childhood
BM failure

t(12;21), t(9;22)

B Cell ALL

Adolescents males thymic masses lymphadenopathy splenomegaly NOTCH-1 (70%)

T Cell ALL

MCC of agranulocytosis

Drug Toxicity

Histologic findings in ALL

LYMPHOBLASTS - scant basophilic cytoplasm and larger nuclei Hypercellular marrow with LYMPHOBLASTS PAS (+)

Unique metastatic sites of ALL

CNS testis

Neoplastic proliferation of myeloid precursor >20% myeloblasts in BM

AML FAB M3 - BEST prognosis Therapy related - POOR prognosis

Peculiar manifestations of AML

DIC (APML) Mucocutaneous deposits (AML w/ monocytic differentiation) Myeloid sarcoma (soft tissue mass)

Acute Promyelocytic Leukemia (APML) associated with DIC

t (15;17) AUER RODS - needle like azurophilic granules in cytoplasm FAGGOT CELLS - w/ numerous Auer rods

MC leukemia of adults in WESTERN world AUTOIMMUNE constitutional symptoms hepatosplenomegaly, lymphadenopathy immune abnormalities: infection or AIHA or ITP

CLL/SLL CLL > 5000 SLL <5000

Histologic findings of CLL/SLL

SHEETS OF SMALL LYMPHOCYTES PROLIFERATION CENTERS - pathognomonic SMUDGE CELLS - disrupted cells from smearing

Notable sequela of CLL

RICHTER SYNDROME - progression to DLCBL

BCR-ABL t(9;22) Philadelphia chromosome splenomegaly (extramedullary hematopoiesis) ACCELERATED PHASE - worsening anemia or thrombocytopenia with BASOPHILIA BLAST CRISIS - resembles acute leukemia

CML

Morphology of CML

HYPERCELLULAR MARROW W/ SEA BLUE HISTIOCYTES WBC >100 000/mm3 LOW leukocyte alkaline phosphatase (LAP) blast <10% THROMBOCYTOSIS

3 PHASES OF CML

CHRONIC phase ACCELERATED phase *10-19% *increased WBC count (unresponsive to therapy) *increased splenomegaly BLAST crisis *>20% *CHLOROMA - solid focus of leukemia outside BM

Danger signs of CML

INCREASED blast count INCREASED basophil count DECREASED platelet count

Single AXIAL group of nodes CONTIGUOUS painless lymphadenopathy LN --> spleen --> liver --> BM fever, night sweats, weight loss

HODGKIN LYMPHOMA nodular sclerosis - MC! mixed cellularity lymphocyte rich lymphocyte depletion - least common; worst prognosis; PLHIV nodular lymphocyte predominance - young adult

Fundamental molecular abnormality in HODGKIN LYMPHOMA

NF-kB activation

The most important prognostic variable in Hodgkin Lymphoma

STAGE

Treatment for Hodgkin Lymphoma

Radiotherapy Chemotherapy Anti-CD30 (Brentuxumab) Immune Checkpoint Inhibitors

MULTIPLE PERIPHERAL NODES NON - CONTIGUOUS (+) mesenteric nodes and Waldeyer ring involvement (+) extranodal presentation

NON - HODGKIN LYMPHOMA

NON HODGKIN LYMPHOMA PERIPHERAL B CELL LYMPHOMAS SMALL CELL LYMPHOCYTIC PROLIFERATION

Small lymphocytic lymphoma Mantle cell lymphoma Follicular lymphoma Marginal zone lymphoma

NON HODGKIN LYMPHOMA PERIPHERAL B CELL LYMPHOMAS AGGRESSIVE B CELL LYMPHOMAS

Diffuse large B cell lymphoma (DLCBL) Burkitt lymphoma

PERIPHERAL T CELL AND NK CELL LYMPHOMAS

Anaplastic large cell lymphoma Mycosis fungoides/Sezary syndrome

MC Non Hodgkin Lymphoma

Diffuse large B cell lymphoma (DLCBL)

Associated w/ LATENT EBV infection HIGH MITOTIC INDEX + NUMEROUS APOPTOTIC CELLS STARRY SKY PATTERN royal blue cytoplasm + clear cytoplasmic vacuoles

BURKITT LYMPHOMA t(8;14) ONE OF THE FASTEST GROWING HUMAN TUMOR

splenomegaly pancytopenia and DRY TAP on BM aspiration monocytopenia HAIR-LIKE PROJECTIONS

HAIRY CELL LEUKEMIA (HCL)

MC indolent lymphoma of adults painless generalized lymphadenopathy follicular architecture with uniformly sized follicles with thinned mantle zones CENTROBLASTS AND CENTROCYTES

FOLLICULAR LYMPHOMA BCL2; t(14;18)

Peripheral B cell lymphomas that arise in sites of CHRONIC INFLAMMATION

MARGINAL ZONE LYMPHOMA H.pylori - stomach Sjogren syndrome - salivary gland Hashimoto thyroiditis - thyroid gland

Most important plasma cell neoplasm

Multiple Myeloma

65-70 years Clinical features: CRAB HyperCalcemia (increased secretion of cytokines [eg, IL-1, TNF-a, RANK-L] by malignant plasma cells osteoclast activity) Renal involvement Anemia Bone lytic lesions (“punched out” on X-ray A ) back pain

MULTIPLE MYELOMA vertebral column

Histologic findings in Multiple Myeloma

plasmacytosis plasmablasts bizarre multinucleated giant cells FLAME CELLS MOTT CELLS - with multiple grapelike cytoplasmic inclusions RUSSELL BODIES - globular CYTOPLASMIC inclusions DUTCHER BODIES - globular NUCLEAR inclusions

Laboratory findings in Multiple Myeloma

ROULEAUX FORMATION Plasma cell leukemia HYPERCALCEMIA MONOCLONAL GAMMOPATHY (IgG) BENCE JONES PROTEINURIA (Ig LIGHT CHAIN)

Laboratory findings in Multiple Myeloma

ROULEAUX FORMATION Plasma cell leukemia HYPERCALCEMIA MONOCLONAL GAMMOPATHY (IgG) BENCE JONES PROTEINURIA (Ig LIGHT CHAIN)

MCC of death in multiple myeloma

INFECTIONS Renal failure